Case Presentation

• 3 days old Baby boy of Mrs.R from Kottawa presented with yellowish discoloration of the body and eyes for 1 day duration

Antenatal history• This is mothers second pregnancy with an uneventful

antenatal period upto 34th week where the OGTT test has showed increased glucose levels but was controlled on diet.

Birth history• Baby was delivered via a normal vaginal delivery at 37

weeks.• Birth weight was 2.7Kg.• APGAR 1 min- 9 , 5 min- 10• Blood sugars were monitored 4 hrly for 24 hrs and was

above 46mg.

History of presenting complaint

• Patient was last well 1 day ago where the mother has noticed yellowish discolouration of the whole body and sclera.

• During the first two days stools were said to be dark in colour which has turned yellowish by the 3rd day but it was not pale.

• Mother also mentioned of 1 episode of dark coloured urine on the 3rd day after birth.

• Baby was said to be lethargic where the mother had to wake him up during the feeds.

• No difficulty in feeding , excessive crying, fever or discharge from the umbilicus noted.

• Urine output, Bowel output normal.

• In the evening of the 3rd day after birth baby was brought to the hospital and since the baby was severly jaundiced, blood was sent for FBC,CRP,SBR,GP,DCT and started on triple phototherapy. After 1 hr serum bilirubin level was found out to be 26.46mg/dl which was above the exchange transfusion level.

• Blood group - mother is O +ve Fathers not known baby is B +ve

• Not a consangous marriage.

• Immunization historyBCG given within 24hrs.

• Family historyNo family members with jaundice in the neonatal period. No history of anemia, splenectomy or Bile stones in family members or known heredity for haemolytic disorders.

• Social historyBaby is living with his mother, father , grandmother and the elder sibling who is 1 ½ years.Mother is 36 years old and a housewife.Father is a 36 years old businessman.She receives a good family supportThe family is financially stable.

Examination• Baby was on triple phototherapy in NICU.• No dysmorphic features ,not dyspnoeic. • Yellow discolouration of the skin and sclera

was observed.• Weight on examination 2.61kg (only 4% loss

not significant)• OFC 32cm.• Length 46cm

• BP-64/29• HR – 152 BPM• CRFT - < 2 sec• RR – 42• TEMP – 98.4 F

• Other than that Neonatal examination was unremarkable.

• Management

Soon after admission – Triple phototherapy started • 10% Dextrose infusion at 6.8CC per hour• EBM 30CC per 3 hours.

• Investigations doneFBC/CRP/SBR/GP/ DCT/ Blood picture/ Reticulocyte count

22 Aug 2016 ( day 1 of admission)– FBC - Hb 12.9 g/dl

- RBC 3.34 10^12/L- HCT 37 %- WBC 14.53 10^9/L- N 38.1%- L 42.8%- PLT 343 10^9/L

-- CRP -1.4 mg/L

--SBR Total Bil – 26.46mg/dl Direct Bil – 1.21mg/dl Indirect Bil – 25.25mg/dl

-DCT – Negative-Blood group B +ve

• Bilirubin level was above the exchange transfusion level.

• Preparation for an exchange transfusion was done which is to be carried out after the next serum bilirubin level in 8 hrs.

• While waiting, two hours after admission 40ml of group B FFP at 13.3CC per hour over 3 hours IV Furosemide 2.5mg

23 Aug 2016 (2nd day of admission)• SBR at 5 a.m

Total Bil – 20.06mg/dlDirect Bil – 1.73mg/dlIndirect Bil – 18.33mg/dl

• Bilirubin level below exchange transfusion level

No exchange transfusion done• Triple photo therapy continued• IV fluids stopped and EBM given.

• Reticulocyte count at 10 a.m - 10%

• FBC at 6 p.m- Hb 11.6 g/dl

- RBC 2.99 10^12/L- HCT 32.4%- WBC 15.91 10^9/L- N 41.7%- L 39.8%- PLT 356 10^9/L

SBR at 6 p.mTotal Bil – 16.55mg/dlDirect Bil – 1.59mg/dlIndirect Bil – 14.96mg/dl

Triple phototherapy continued

24 Aug 2016 (3rd day of admission)

• Total Bilirubin at 8 a.m13.1mg/dl

• Blood pictureRBC – Normochromic Macrocytic red cells

Occational sphyrocytes, tear drops, contracted cells and basophilic stipling.

PolychromasiaWBC – Neutrophilia. NO abnormal cellsPLT – Plentiful

• Transferred to paediatric ward from NICU • Changed Triple phototherapy to single

phototherapy

25 Aug 2016 (4th day of admission)• FBC at 7 am

- Hb 11.6 g/dl

- RBC 3.14 10^12/L- HCT 33.4%- WBC 14.4 10^9/L- N 35%- L 48%- PLT 382 10^9/L

• Total Bilirubin at 9 am – 15.06mg/dl

• Single phototherapy continued

26th Aug 2016 (5th day of admission)

• SBR at 7 a.mTotal Bil – 15.96mg/dlDirect Bil – 1.07mg/dlIndirect Bil – 14.89mg/dl

Single phototherapy continued

Bilirubin levels of the patient

Bilirubin levels and treatment during the admission

22/08/2016Day 1

23/08/2016Day 2

24/08/2016Day 3

25/08/2016Day 4

26/08/2016Day 5

Total Bilirubin(mg/dl)

26.46 AM PM 13.10 15.06 15.96

20.06 16.55

Direct Bilirubin (mg/dl)

1.21 1.73 1.59 - - 1.07

Indirect Bilirubin(mg/dl)

25.25 18.33 14.96 - - 14.89

FFP +IV Frusemide(9.30 p.m)

Triple phototherapy Single phototherapy

On Discharge

• Serum bilirubin level was several squares below photo therapy level.

• Very mild jaundice was present.• Baby was active and feeding well

Neonatal Jaundice

Yellowish discoloration of skin and scleraClinically jaundiced when bilirubin level

reach about 80µmol/ l

• Usually over 50% of all new born infants become visibly jaundiced because of,

High [Hb] at birth RBC breakdown RBC life span of infants are short(70 days) Less efficient bilirubin metabolism

Early neonatal jaundice is important :# as it may be a sign of another disorder;

Eg: Haemolytic anaemia Infection Metabolic disease Liver disease# Unconjugated bilirubin deposition in basal ganglia may

cause Kernicterus

In the neonate, defect in any of the above steps can give rise to problems

Bilirubin metabolism

Types of Jaundice A. Physiological Jaundice • Jaundice can be seen in 60% of Term infants & 80% of Preterm

infants.• It is mostly physiological

Features of physiological jaundice: (ALL of the following) Jaundice that first appears between 24-72 hours of age Maximum intensity is seen on 4-5th day in term and 7th day in

preterm neonates Usually mild and less than 15mg/dl Clinically undetectable after 14 days

Physiological jaundice is a diagnosis by exclusion.no treatment is required but baby should be observed closely for signs of worsening jaundice.

B. Pathological Jaundice

Features of pathological jaundice: ( ANY of the following)

Clinical jaundice within 24 hours of birth Total serum bilirubin (TSB) increase by >5mg/dl/day

(85µmol/l/day) OR 0.5mg/dl/hour (8.5µmol/l/hour) Conjugated serum bilirubin >20% of total serum

bilirubin level. Clinical jaundice persisting for > 2 weeks* in full term

and >3 weeks in preterm neonates (prolong jaundice). (*NB : except in cases of breast milk jaundice)

Causes of Jaundice• Hyperbilirubinaemia in the first week of life is usually of the

unconjugated (Indirect) variety.• Although conjugated hyperbilirubinaemia (Direct) occurs less

commonly , it is always pathological.

Appearing within 24 hours of age ; Haemolytic disease of newborn – Blood group incompatibility - ABO ,Rh and minor blood group incompatibility Hereditary hemolytic anaemias - Congenital spherocytosis G6PD deficiency Infections –perinatal sepsis

Appearing after 24 hours after life ;All of the abovePhysiologicalPolycythemiaConcealed haemorrhages- Subneurotic

/subarachnoid/intraventricular haemorrhage

Prolonged jaundice (In term babies > 2 weeks) (In pre term babies > 3 weeks)

A.Prolong Unconjugated hyperbilirubinaemia: New or persisting sepsis- eg: UTI Metabolic –Hypothyroidism , Galactosaemia Persisting haemolysis Breast milk jaundice

B. Prolonged Conjugated hyperbilirubinaemia: Neonatal hepatitis - Congenital infections Intrauterine infections (TORCH) α1 antitrypsin deficiancy Extra hepatic biliary atresia Choledochal cyst Metabolic disorders- Eg: Galactocaemia Crigler Najjar Syndrome

Assessment of a jaundiced within the 1st week

• This is directed towards assessing the severity, complications and determining the aetiology of jaundice.

Severity of jaundice

• Jaundice in the newborn progresses in the cephalo-caudal direction and thus the extent of yellowness of the skin is useful to assess the level of bilirubin.

• When a neonate is clinically jaundiced, the TSB is usually >5 mg/dl (85 μmol/l).

• Serum bilirubin profiles and transcutaneous methods• Kramer’s criteria are used to clinically estimate severity

Kramer’s Criteria

1 or less - >10mg/dl2 or less - >15mg/dl5 – definitely need interventions

These values are not absolute

Management• Mx of jaundice is directed towards reducing the level of bilirubin

and preventing CNS toxicity.

1. Reduction of bilirubin is achieved by phototherapy and / or exchange transfusion.

2. Dilution of bilirubin by giving FFP, fluids. 3. Hyperbilirubinaemia due to dehydration may be prevented by

early and frequent feeding. The decision to treat depends on the severity and the cause of

jaundice.

Phototherapy

Preparation for phototherapy• This involves exposure of the naked baby to blue-green

spectrum of wave length 450-460nm• kept about 18 inches away from the light.• The light waves convert the bilirubin to water soluble

nontoxic forms which are then easily excreted.• Frequent feeding, every 2-3 hours and change of posture

should be promoted in an infant receiving phototherapy.• Eye shades should be fixed.• External genitalia should be covered

Provision of phototherapy Degree of phototherapy depends on severity of jaundice

Initiate continuous multiple phototherapy if any of the following apply;

• TSB level is rising> 0.5 mg/dl/hr• TSB is at a level within 3mg/dl below the level for which exchange

transfusion is indicated• TSB level fails to respond to single phototherapy

• When bilirubin level falls during continuous multiple phototherapy to a level > 3mg/dl below the threshold level for which exchange transfusion is indicated , step down.

Repeat serum bilirubin measurement 4–6 hours after initiating phototherapy and continue 6hrly if rising or non responsive or 12hrly if responsive.

Side effects of phototherapy• Increased insensible water loss when providing phototherapy

in cots: breastfeed more frequently / provide adequate fluids to avoid dehydration

• Loose green stools: weigh often and compensate with breast milk.

• Skin rashes: harmless, no need to discontinue phototherapy;

• Bronze baby syndrome: occurs if baby has conjugated hyperbilirubinaemia. If so, discontinue phototherapy

• Hypo or hyperthermia: monitor temperature frequently.

• Damage to eyes: cover eyes

Exchange Transfusion• should be performed if the TSB remains in

exchange transfusion range as per treatment threshold graphs, despite effective phototherapy.

• Immediate exchange transfusion is indicated if features of bilirubin encephalopathy are evident.

• Delay in treatment may result in permanent brain damage.

• When referring a baby with jaundice, make sure that either the mother is referred or mother’s blood sample is sent.

Use a double-volume exchange transfusion (2 x 80 ml /kg)

Umbilical vessels are the preferred access method for performing an exchange transfusion

Electrolytes, blood gases and vital signs should be monitored during exchange transfusion

Type of blood• In ‘Rh’ isoimmunisation, the best choice would be O

negative packed cells suspended in AB positive plasma. O negative whole blood or cross-matched baby’s blood group (but Rh negative) may also be used.

• For ‘ABO’ isoimmunisation, O group (Rh compatible) packed cells suspended in AB plasma or O group whole blood (Rh compatible with baby) should be used.

• In other situations baby’s blood group should be used. All blood must be cross matched against maternal plasma.

complications of exchange transfusion

anaphylaxis ABO incompatibility Electrolyte disturbances Acid base disturbances Hypothermia Infection

Thank you