not so well, well baby

7/30/2019 Not so well, well baby

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Morning Report

The “not so well” well baby Katherine MacDonald, PGY2



History

• Term AGA 2475 gram male born to at 20 yoG1P0 at 37 weeks gestation via induced vaginaldelivery due to mild maternal pre eclampsia and

a non reactive non-stress test. APGARS of 9 and9. WDS

• Nuchal cord x1, Clear fluid, and no apparentinfant complications .

• Prenatal US of L pelviectasis



Family/Social History

• No family history hyperbilirubinemia, congenitalheart disease, or children with multiple

surgeries. Maternal grandmother had asthma asa child

• Mother lives with her partner and his mother

and father. Her partner is currently in jail due to breaking probation and drug use. She isunemployed. She has a history of drug use andreports that she has not used for 3 years.



Physical ExamGeneral: Decreased activity and responsiveness to painful stimuli HEENT: Normal Shape, AFOSF, RR not assessed. Ears nml shape.

Nose patent. Palate intact. No neck masses, Nml clavicles.

Respiratory: Normal Effort, CTAB, good aeration

Cardiac: CRT< 3 seconds, RRR, Murmur (Grade II, Location: SEM,LUSM, nonradiating), Brachial & femoral pulses palpable & equal.

Abdomen: BSP, Soft, non tender, Normal umbilicus, No HSM

External genitalia: Normal male, testes descended.

Anus: patent.

Back: Back was straitExtremities/Hips: Normal digits, no hip click

Neurological: decreased tone and activity

Skin: No rashes, birth marks, petechiae, bruising or vesicles



What are you to do???

You are just about to start rounds when the nurse

tells you that the baby’s blood glucose is

< 30 and has a low temp…..

Confirmed blood glucose <5!!



Labs:• CBC: wbc 10.8 (N58, B9, L30, M 7),

hgb 17 hct 53 plt 87 ->70->37. I:T0.16

• BMP: Na 138 K 4.5 Cl 107 CO2 19BUN 6 Cr 0.84 Gluc 20

• LFTs: AST 104 (hemolyzed), ALT 13, Alk Phos 132 Total protein 5.7

• Bili at ~ 24 H 7.2 (LL 8)

• CBG: 7.43/35.4/23.1/45/-0.1

• PT 16.4 PTT 33 Fibrinogen 199 DD2.9

• CRP 1.9

• Lumbar Puncture: WBC 675, RBC 306, 750Diff: 1 band, 67N 18L 14M, Glucose 31Protein 450

• Blood and Spinal fluid Clx neg

• Serum HSV neg

• Urine CMV neg

• Mec Tox neg

• NMS normal

• Serum Cortisol 4.3

• CXR: Scattered perihilar atelectasis

• ECHO: Small PDA



NEONATAL TRANSIENT HYPOGLYCEMIA

• Inadequate Substrate or Immature EnzymeFunction : Prematurity, SGA, Normal newborn

• Transient , Neonatal Hyperinsulinism Presentin: Infant of diabetic mother, SGA, Discordanttwin, Birth asphyxia, Infant of toxemic mother



NEONATAL, INFANTILE, OR CHILDHOODPERSISTENT HYPOGLYCEMIAS• Hormonal Disorders: Hyperinsulinism,

channellopathies, and mitochondrialuncoupling., Recessive HADH (hydroxyl acylCoA dehydrogenase) mutation HI, RecessiveUCP2 (mitochondrial uncoupling protein 2)

mutation HI Dominant glucokinase HI,Dominant glutamate dehydrogenase HI(hyperinsulinism/hyperammonemiasyndrome), Dominant mutation in HNF4A (hepatic nuclear factor 4 alpha) HI with MODY later in life, Dominant mutation in SLC16A1(thepyruvate transporter)-exercise-inducedhypoglycemia

▫ Acquired islet adenoma▫ Beckwith-Wiedemann syndrome▫ Insulin administration (Munchausen syndrome

by proxy)▫ Oral sulfonylurea drugs▫ Congenital disorders of glycosylation

• Counter-Regulatory HormoneDeficiency Panhypopituitarism, Isolatedgrowth hormone deficiency, ACTH deficiency,

Addison disease, Epinephrine deficiency • Glycogenolysis and Gluconeogenesis

Disorders: Glucose-6-phosphatase deficiency (GSD 1a), Glucose-6-phosphate translocasedeficiency (GSD 1b), Amylo-1,6-glucosidase(debranching enzyme) deficiency (GSD3), Liver phosphorylase deficiency (GSD 6),Phosphorylase kinase deficiency (GSD 9),Glycogen synthetase deficiency (GSD 0),Fructose-1,6-diphosphatase deficiency,Pyruvate carboxylase deficiency, Galactosemia

Lipolysis Disorders, Fatty Acid OxidationDisorders Carnitine transporter deficiency (primary carnitine deficiency), Carnitinepalmitoyltransferase-1 deficiency Carnitinetranslocase deficiency, Carnitinepalmitoyltransferase-2 deficiency, Secondary carnitine deficiencies, Very long, long-,medium-, short-chain acyl CoA dehydrogenasedeficiency



OTHER ETIOLOGIES• Substrate-Limited ,Ketotic hypoglycemia, Oral hypoglycemic

agents, Insulin, Trimethoprim-sulfamethoxazole (with renal failure)

• Liver Disease Reye syndrome, Hepatitis, Cirrhosis, Hepatoma

• Amino Acid and Organic Acid Disorders Maple syrup urinedisease, Propionic acidemia, Methylmalonic acidemia, Tyrosinosis,Glutaric aciduria 3-Hydroxy-3-methylglutaric aciduria

• Systemic Disorders Sepsis, Carcinoma/sarcoma (secreting—insulin-like growth factor II) Heart failure, Malnutrition,Malabsorption, Anti-insulin receptor antibodies, Anti-insulinantibodies, Neonatal hyperviscosity, Renal failure, Diarrhea, Shock



Neonatal Hypoglycemia

• Epidemiology

• Definition

• Physiology

• Who is at risk

• Differential

• Management



epidemiology

• estimated incidence of symptomatichypoglycemia in newborns is 1-3/1,000

• Several fold increase in incidence in at risk groups



definition

• Debatable

• Symptoms can occur at varying glucoseconcentrations.

• <47? 45? 50?



physiology

• Has to go from 0 -> 60!

• Relied on mom’s blood glucose and the newbornmust transition to sustaining its owns BG

• 3 events must happen:

▫ changes in hormones, in their receptors, & enzyme

activity



physiology cont.

• Insulin level and remains at basal state forseveral days

• There is a SURGE of catecholemines andepinephrine increases growth hormone

• Theses changes glycogenolysis,gluconeogenesis, activate lipolysis andketogenesis



physiology cont.

liver glycogen stores becomerapidly depleted within hoursof birth -> Gluconeogenesis

Increase in FFA and Ketones-> acts as an alternative energy source and is utilized ingluconeogenesis in order reserve

to glucose for the brain

Increase in the enzymes-> esp ones in the rate limiting steps in gluconeogensis



physiology cont.

• Hypoglycemia ->

• hormones do not change appropriately

• reserves not available (hepatic glycogen, musclefor AA, and lipid stores for FA)

• key enzymes not at the appropriate level



Whose at risk?

• SGA, IDM, Preterm and late preterm.

• LGA “controvesial” because it is difficult toexclude gestational DM or pre diabetes with oneGTT



Appropriate screening and

Management



Screening based upon the frequency and duration related to risk factors

Recommend feed within 1 hour ->test glucose 30 after feed

IDM have asymptomatic hypoglycemia:1-2 hours

LGA/SGA: Usually 3 H…Up to 10 days!

Late Preterm/ SGA : pre-prandial for 24 hours

IDM/LGA: Pre-prandial for 12 hours



Management

Don’t delay treatment!

If persistent, broadenthe differential



Differential• Systemic (viral / bacterial

infection, polycythemia)

• Persistent HyperinsulinemicHypoglycemia of Infancy

▫ AR at birth, Usually macrosomic

▫ Mutations in the K channels

• Hyperinsulinemia

▫ Diffuse beta cell hyperplasia

▫ Focal beta cell microadenomas

• Panhypopituitarism

• ACTH or GH deficiency

• Beckwith Wiedemann(hyperinsulinemic state)

• Falciparum Malaria

• “Metabolic”: Galactosemia,MSUD, Type 1 Glycogen

storage disease, Pyruvatedehydrogenase def.



Effects of Hypoglycemia

• Studies are challenging to interpret due to variability

• Transient asymptomatic hypoglycemia notshown to have long term sequele

• Symptomatic hypglycemia can have long term

effects on neurodevelopment▫ 94% abnormal MRI▫ 26/34 at 18 mo had mild to

severe impairment



Effects of hypoglycema

• Study of premature infants and related thepersistence of hypoglycemia to neurodevelopmentaloutcomes▫ If >5 episodes -> lower scores on Bayley

developmental scale at 18 mo and 3.5 fold increase of DD and CP

• Study comparing LGA babies (normo vs

hypoglycemia)▫ No sig diff in DDS or the Child Behavior checklist▫ No overall sig diff in IQ- but those with hypoglycemia

had lower reasoning score subcategory (?chance)



References• Burns, CM, et al. Patterns of Cerebral Injury and Neurodevelopmental

Outcomes After Symptomatic Neonatal Hypoglycemia. Pediatrics Vol. 122No. 1 July 1, 2008 pp. 65 -74 (doi: 10.1542/peds.2007-2822)

• Boluyt, Nicole et al. Neurodevelopment After Neonatal Hypoglycemia: A Systematic Review and Design of an Optimal Future Study Pediatrics Vol.117 No. 6 June 1, 2006 pp. 2231 -2243 (doi: 10.1542/peds.2005-1919)

• Adamkin DH. Clinical Report—Postnatal Glucose Homeostasis in Late-Preterm and Term Infants Pedicatrics Vol 127, No 3, March 2011 pp 575-570 (doi:10.1542/peds.2010-3851)

• Nelson Textbook of Pediatrics 19th Ed. Chapter 86. pp 517-531

not so well, well baby

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