ANAEMIA IN PREGNANCY

Dr SHASHWAT JANI Dr SHASHWAT JANI MS ( OBS - GYN MS ( OBS - GYN ))

Diploma in Advanced laparoscopy Diploma in Advanced laparoscopy Assistant Professor Sheth V S General HospitalAssistant Professor Sheth V S General Hospital

Smt NHL Municipal Medical college AhmedabadSmt NHL Municipal Medical college Ahmedabad

Mobile +91 99099 44160Mobile +91 99099 44160

E- mail drshashwatjanigmailcomE- mail drshashwatjanigmailcom

Commonest haematological dsCommonest haematological ds

40-60 maternal deaths in developing 40-60 maternal deaths in developing countriescountries

Death bo cardiac failure haemorrhage Death bo cardiac failure haemorrhage infection pre-eclampsiainfection pre-eclampsia

Definition Definition

Condition of low circulating Hb in which Condition of low circulating Hb in which Hb concentration has fallen below a Hb concentration has fallen below a threshold lying at 2 standard deviation threshold lying at 2 standard deviation below the median of a healthy population below the median of a healthy population of same age sex stage of pregnancyof same age sex stage of pregnancy

WHO WHO ICMR categorises anaemia depending on ICMR categorises anaemia depending on

Hb levelHb level

Classification of AnaemiaClassification of Anaemia

PhysiologicalPhysiological

PathologicalPathological

Causes of pathological anaemiaCauses of pathological anaemia

Deficiency Deficiency anaemiaanaemia

Haemorrhagic Haemorrhagic anaemia anaemia

Acute chronicAcute chronic

Hereditary Hereditary anaemiaanaemia

Bone marrow Bone marrow insufficiency ndash insufficiency ndash aplasia hypoaplasia hypo

Anaemia of Anaemia of infection- infection- malaria TBmalaria TB

Chronic ds- Chronic ds- renalrenal

Concept of physiological anaemiaConcept of physiological anaemia

Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement

Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation

Iron requirementIron requirement

Menstruating females 1-2mg per dayMenstruating females 1-2mg per day

Pregnant females 15-25mg per dayPregnant females 15-25mg per day

Children 1mg per dayChildren 1mg per day

An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed

Total iron requirement in pregnancyTotal iron requirement in pregnancy

Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday

in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk

Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed

Iron distribution on pregnancyIron distribution on pregnancy

Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg

Iron distribution in bodyIron distribution in body

Total iron 3-5gTotal iron 3-5g

Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g

Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g

Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g

Plasma iron 3-4mgPlasma iron 3-4mg

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Commonest haematological dsCommonest haematological ds

40-60 maternal deaths in developing 40-60 maternal deaths in developing countriescountries

Death bo cardiac failure haemorrhage Death bo cardiac failure haemorrhage infection pre-eclampsiainfection pre-eclampsia

Definition Definition

Condition of low circulating Hb in which Condition of low circulating Hb in which Hb concentration has fallen below a Hb concentration has fallen below a threshold lying at 2 standard deviation threshold lying at 2 standard deviation below the median of a healthy population below the median of a healthy population of same age sex stage of pregnancyof same age sex stage of pregnancy

WHO WHO ICMR categorises anaemia depending on ICMR categorises anaemia depending on

Hb levelHb level

Classification of AnaemiaClassification of Anaemia

PhysiologicalPhysiological

PathologicalPathological

Causes of pathological anaemiaCauses of pathological anaemia

Deficiency Deficiency anaemiaanaemia

Haemorrhagic Haemorrhagic anaemia anaemia

Acute chronicAcute chronic

Hereditary Hereditary anaemiaanaemia

Bone marrow Bone marrow insufficiency ndash insufficiency ndash aplasia hypoaplasia hypo

Anaemia of Anaemia of infection- infection- malaria TBmalaria TB

Chronic ds- Chronic ds- renalrenal

Concept of physiological anaemiaConcept of physiological anaemia

Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement

Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation

Iron requirementIron requirement

Menstruating females 1-2mg per dayMenstruating females 1-2mg per day

Pregnant females 15-25mg per dayPregnant females 15-25mg per day

Children 1mg per dayChildren 1mg per day

An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed

Total iron requirement in pregnancyTotal iron requirement in pregnancy

Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday

in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk

Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed

Iron distribution on pregnancyIron distribution on pregnancy

Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg

Iron distribution in bodyIron distribution in body

Total iron 3-5gTotal iron 3-5g

Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g

Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g

Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g

Plasma iron 3-4mgPlasma iron 3-4mg

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Definition Definition

Condition of low circulating Hb in which Condition of low circulating Hb in which Hb concentration has fallen below a Hb concentration has fallen below a threshold lying at 2 standard deviation threshold lying at 2 standard deviation below the median of a healthy population below the median of a healthy population of same age sex stage of pregnancyof same age sex stage of pregnancy

WHO WHO ICMR categorises anaemia depending on ICMR categorises anaemia depending on

Hb levelHb level

Classification of AnaemiaClassification of Anaemia

PhysiologicalPhysiological

PathologicalPathological

Causes of pathological anaemiaCauses of pathological anaemia

Deficiency Deficiency anaemiaanaemia

Haemorrhagic Haemorrhagic anaemia anaemia

Acute chronicAcute chronic

Hereditary Hereditary anaemiaanaemia

Bone marrow Bone marrow insufficiency ndash insufficiency ndash aplasia hypoaplasia hypo

Anaemia of Anaemia of infection- infection- malaria TBmalaria TB

Chronic ds- Chronic ds- renalrenal

Concept of physiological anaemiaConcept of physiological anaemia

Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement

Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation

Iron requirementIron requirement

Menstruating females 1-2mg per dayMenstruating females 1-2mg per day

Pregnant females 15-25mg per dayPregnant females 15-25mg per day

Children 1mg per dayChildren 1mg per day

An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed

Total iron requirement in pregnancyTotal iron requirement in pregnancy

Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday

in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk

Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed

Iron distribution on pregnancyIron distribution on pregnancy

Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg

Iron distribution in bodyIron distribution in body

Total iron 3-5gTotal iron 3-5g

Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g

Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g

Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g

Plasma iron 3-4mgPlasma iron 3-4mg

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Classification of AnaemiaClassification of Anaemia

PhysiologicalPhysiological

PathologicalPathological

Causes of pathological anaemiaCauses of pathological anaemia

Deficiency Deficiency anaemiaanaemia

Haemorrhagic Haemorrhagic anaemia anaemia

Acute chronicAcute chronic

Hereditary Hereditary anaemiaanaemia

Bone marrow Bone marrow insufficiency ndash insufficiency ndash aplasia hypoaplasia hypo

Anaemia of Anaemia of infection- infection- malaria TBmalaria TB

Chronic ds- Chronic ds- renalrenal

Concept of physiological anaemiaConcept of physiological anaemia

Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement

Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation

Iron requirementIron requirement

Menstruating females 1-2mg per dayMenstruating females 1-2mg per day

Pregnant females 15-25mg per dayPregnant females 15-25mg per day

Children 1mg per dayChildren 1mg per day

An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed

Total iron requirement in pregnancyTotal iron requirement in pregnancy

Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday

in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk

Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed

Iron distribution on pregnancyIron distribution on pregnancy

Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg

Iron distribution in bodyIron distribution in body

Total iron 3-5gTotal iron 3-5g

Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g

Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g

Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g

Plasma iron 3-4mgPlasma iron 3-4mg

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Causes of pathological anaemiaCauses of pathological anaemia

Deficiency Deficiency anaemiaanaemia

Haemorrhagic Haemorrhagic anaemia anaemia

Acute chronicAcute chronic

Hereditary Hereditary anaemiaanaemia

Bone marrow Bone marrow insufficiency ndash insufficiency ndash aplasia hypoaplasia hypo

Anaemia of Anaemia of infection- infection- malaria TBmalaria TB

Chronic ds- Chronic ds- renalrenal

Concept of physiological anaemiaConcept of physiological anaemia

Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement

Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation

Iron requirementIron requirement

Menstruating females 1-2mg per dayMenstruating females 1-2mg per day

Pregnant females 15-25mg per dayPregnant females 15-25mg per day

Children 1mg per dayChildren 1mg per day

An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed

Total iron requirement in pregnancyTotal iron requirement in pregnancy

Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday

in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk

Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed

Iron distribution on pregnancyIron distribution on pregnancy

Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg

Iron distribution in bodyIron distribution in body

Total iron 3-5gTotal iron 3-5g

Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g

Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g

Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g

Plasma iron 3-4mgPlasma iron 3-4mg

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Concept of physiological anaemiaConcept of physiological anaemia

Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement

Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation

Iron requirementIron requirement

Menstruating females 1-2mg per dayMenstruating females 1-2mg per day

Pregnant females 15-25mg per dayPregnant females 15-25mg per day

Children 1mg per dayChildren 1mg per day

An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed

Total iron requirement in pregnancyTotal iron requirement in pregnancy

Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday

in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk

Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed

Iron distribution on pregnancyIron distribution on pregnancy

Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg

Iron distribution in bodyIron distribution in body

Total iron 3-5gTotal iron 3-5g

Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g

Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g

Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g

Plasma iron 3-4mgPlasma iron 3-4mg

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Iron requirementIron requirement

Menstruating females 1-2mg per dayMenstruating females 1-2mg per day

Pregnant females 15-25mg per dayPregnant females 15-25mg per day

Children 1mg per dayChildren 1mg per day

An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed

Total iron requirement in pregnancyTotal iron requirement in pregnancy

Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday

in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk

Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed

Iron distribution on pregnancyIron distribution on pregnancy

Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg

Iron distribution in bodyIron distribution in body

Total iron 3-5gTotal iron 3-5g

Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g

Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g

Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g

Plasma iron 3-4mgPlasma iron 3-4mg

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Total iron requirement in pregnancyTotal iron requirement in pregnancy

Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday

in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk

Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed

Iron distribution on pregnancyIron distribution on pregnancy

Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg

Iron distribution in bodyIron distribution in body

Total iron 3-5gTotal iron 3-5g

Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g

Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g

Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g

Plasma iron 3-4mgPlasma iron 3-4mg

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Iron distribution on pregnancyIron distribution on pregnancy

Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg

Iron distribution in bodyIron distribution in body

Total iron 3-5gTotal iron 3-5g

Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g

Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g

Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g

Plasma iron 3-4mgPlasma iron 3-4mg

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Iron distribution in bodyIron distribution in body

Total iron 3-5gTotal iron 3-5g

Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g

Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g

Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g

Plasma iron 3-4mgPlasma iron 3-4mg

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)

Cereals seeds veg milk eggCereals seeds veg milk egg

Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol

Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate

Absorption varies 2-100Absorption varies 2-100

Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb

myoglobinmyoglobin

Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30

Iron deficiency 50-90Iron deficiency 50-90

Not affected by inhibitorsNot affected by inhibitors

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Erythropoiesis Erythropoiesis

For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed

Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6

riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Causes of iron deficiency anaemiaCauses of iron deficiency anaemia

Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Clinical Features Clinical Features

SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle

weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema

Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Systemic changesSystemic changes

CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state

(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)

Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur

ventricular dilatationventricular dilatation

heart failureheart failure

Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart

ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl

normal QRS Wavenormal QRS Wave

depression of ST segmentdepression of ST segment

Flattening inversion of T Wave Flattening inversion of T Wave

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness

bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale

papillodema ndashrare papillodema ndashrare

bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )

bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria

bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Effect of anaemia on pregnancyEffect of anaemia on pregnancy

Mild anaemia Mild anaemia

No effect except No effect except darriron storedarriron store

Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance

Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Investigation Investigation darrdarrHb conc is late featureHb conc is late feature

Red cell indicesRed cell indices

Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature

Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator

darr darrMCVMCV

darr darrMCHMCH

darr darrMCHCMCHC

SFerritinSFerritin stored iron stored iron

normal 15-300normal 15-300μμglgl

lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency

not affected by recent iron not affected by recent iron ingestioningestion

SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)

TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)

Transferrin sat lt15 (30)Transferrin sat lt15 (30)

Indicate iron deficiency anaemiaIndicate iron deficiency anaemia

Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr

Serum transferrin receptorSerum transferrin receptor

sensitive and specific markersensitive and specific marker

cellular iron statuscellular iron status

level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency

Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk

diagnosis of aplastic anaemiadiagnosis of aplastic anaemia

kala azarkala azar

Depleted store darrferritinDepleted store darrferritin

Iron deficiency with no anaemia Iron deficiency with no anaemia

darr darr ferritinferritin

darr darr transferrin sattransferrin sat

uarr uarrFEPFEP

Anaemia with iron deficiency Anaemia with iron deficiency

darr darrHb red cell indicesHb red cell indices

darr darr transferrin sattransferrin sat

uarr uarrTIBC FEPTIBC FEP

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Normal Blood FilmNormal Blood Film

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

MICROCYTESMICROCYTES

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

HYPOCHROMIAHYPOCHROMIA

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

COMPLICATIONCOMPLICATION

During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery

Labour Labour

bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia

PuerperiumPuerperium

bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Risk period Risk period 30-32wk pregnancy30-32wk pregnancy

During laborDuring labor

Immediately following deliveryImmediately following delivery

Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW

IUDIUD

Preterm babyPreterm baby

SGASGA

uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality

IUGRIUGR

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

TREATMENTTREATMENT

PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth

-Dietary prescription-Dietary prescription

-Food fortification-Food fortification

-Supplement iron therapy-Supplement iron therapy

100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for

minimum 100 Daysminimum 100 Days

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Curative Curative

bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor

bull Iron therapyIron therapy --oral oral

- parentral- parentral

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store

120-240mgd no iron store120-240mgd no iron store

Oral ironOral iron Elemental doseElemental dose

Ferrous sulphateFerrous sulphate 6565

FSulphate(exsiccated)FSulphate(exsiccated) 6565

Ferrous gluconateFerrous gluconate 3636

Ferrous fumarateFerrous fumarate 6767

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort

nausea vomitingnausea vomiting

Slow release preparationSlow release preparation expansive no SE expansive no SE

Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged

uarr uarrdose required to achieve given responsedose required to achieve given response

Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Drawback Drawback Intolerance Intolerance

unpredictable abs rateunpredictable abs rate

Siron restored but difficult to replenish Siron restored but difficult to replenish

iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being

uarr uarrAppetiteAppetite

Improved outlookImproved outlook

haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption

failure to take iron infectionfailure to take iron infection

conc Blood loss folate def conc Blood loss folate def

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Parentral iron therapyParentral iron therapy

IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N

IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj

Tt completed in a dayTt completed in a day

TDI amp Undiluted TDI amp Undiluted

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose

-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent

-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)

SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction

Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline

Undiluted preparationUndiluted preparation given in single dosegiven in single dose

Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly

Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran

Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly

cleared from bodycleared from body

30excreted unchanged30excreted unchanged

SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron

DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma

HO previous reactionHO previous reaction

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Iron Dextran Adverse Reactions

1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia

1048721Increased incidence of these effects withtotal dose infusions

1048721Onset is 24-48 hours after administration

1048721Effects subside within 3-4 days

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Sodium Ferric Gluconate(Ferrlicit) Dose

1048721Give 10ml (125mg elemental iron) during

a total of 8 consecutive dialysis sessions

for a total dose of 1000mg elemental iron

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Sodium Ferric GluconateAdministration

1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes

1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)

1048721Undiluted as a slow IV injection at a rate up to 125mgmin

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours

1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Iron Sucrose (Venofer) Dose1048721

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing

1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc

BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

ANC CARE

lt30WK 30-36WK gt36WK

IRON DEF FOLIC ACID DEF

ORAL IRON

INTOLERANCENON COMLIANCE

IM IV

FOLIC ACID

IRON DEF FOLIC ACID DEF

PARENTRAL

IMIV

ORAL FOLATE

BLOOD TRANSFUSION

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position

- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross

match)match)

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum

3rd stage 3rd stage active managementactive management

4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Megaloblastic anaemiaMegaloblastic anaemia

Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis

Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both

Vit B12 def rare in pregnancyVit B12 def rare in pregnancy

Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Causes of Folic acid deficiencyCauses of Folic acid deficiency

bull Inadequate intakeInadequate intake nausea anorexianausea anorexia

dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

darr darr AbsorptionAbsorption

intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand

twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage

liver dsliver ds vit C defvit C def

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to

methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine

Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara

uarr uarrmultiple pregnancymultiple pregnancy

Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY

Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms

methycobalaminemethycobalamine

adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source

meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia

Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity

(vit B12 def) (vit B12 def)

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl

MCV gt96flMCV gt96fl

MCH gt33pgMCH gt33pg

MCHC normalMCHC normal

Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate

(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency

PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Macrocytic anemiaMacrocytic anemia

The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Effect of folic acid deficiencyEffect of folic acid deficiency

Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia

Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g

im every monthim every month

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Haemoglobinopathies Haemoglobinopathies

Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia

Hb E Hb DHb E Hb D

DIAGNOSIS DIAGNOSIS

Hb electrophoresisHb electrophoresis

Molecular techniqueMolecular technique

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

SICKLE CELL ANAEMIASICKLE CELL ANAEMIA

Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate

replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling

Sickle ds

HOMOZYGOUSHbSS

HETEROZYGOUSHbAS

HbSC

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy

Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia

-Identified by sickling test-Identified by sickling test

-Persistent leucocytosis-Persistent leucocytosis

-uarrSIron-uarrSIron

- Electrophoresis- Electrophoresis

Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental

infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection

uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

MANAGEMENTMANAGEMENT

role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea

- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration

-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic

-Hydration-Hydration

-Contraception (avoid IUCD)-Contraception (avoid IUCD)

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

Thalassemia Thalassemia

Common genetic disorderCommon genetic disorder

darrdarrrate of globin synthesisrate of globin synthesis

Two type Two type

αα Thalassemia Thalassemia

ββ Thalassemia Thalassemia

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types

1gene mut1gene mutbullNo clinical labNo clinical lab

abn abn bullSilent carrierSilent carrier

2 gene mut2 gene mutbullαα thalassemia thalassemia

minorminorbullUnrecognised Unrecognised

3 gene mut3 gene mutbullHbHHbHbullOne functional One functional

GeneGenebullHb unstable dtHb unstable dt

ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia

All 4 geneAll 4 genebullNO NO functional functional

genegenebullIncompatible Incompatible

with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia

Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

THANK

YOU

YOU

ββ thalassemia thalassemia

ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)

homozygoushomozygous

Erythropoietin ineffectiveErythropoietin ineffective

HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F

MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

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MANAGEMENTMANAGEMENT

αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term

blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life

expectancy daily folate expectancy daily folate

suppl pregnancy- 5mgdsuppl pregnancy- 5mgd

ββ thalassemia thalassemiaMajor folate supplMajor folate suppl

Iron CIIron CI

Minor oral Iron amp folateMinor oral Iron amp folate

parentral iron CIparentral iron CI

THANK

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YOU

YOU

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THANK

YOU

YOU