Neonatal Jaundice

WHAT IS KERNICTERUS AND WHAT IS THE CLASSIC TETRAD OF

KERNICTERUS?
Chronic bilirubin encephalopathy, irreversible
Tetrad:
Choreoathetoid cerebral palsy
High frequency central hearing loss
Palsy of vertical gaze
Dental enamel hypoplasia
WHAT ARE SIGNS OF ACUTE BILIRUBIN ENCEPHALOPATHY?
Early:
Alteration in tone of extensor muscles(hypo or hypertonia)
Retrocollis (arching of neck)
Opisthotonus (arching of back)
Poor suck
Shrill cry
Irritability
Lethargy
Fever
Advanced:
Cessation of feeding
Bicycling movements
Irritability
Seizures
Fever
Altered LOC
HOW IS BILIRUBIN PRODUCED AND EXCRETED BY THE NEONATE?
Produced by the breakdown of hemoglobin (hemoglobin is degraded by heme oxygenase
releasing iron, CO and biliverdin; biliverdin converted o bilirubin by biliverdin reductase)
Unconjugated bilirubin bound by albumin in blood stream
Unconjugated bili taken up by liver and conjugated by UDPGT (uridine diphophate
glucuronosyltransferase)
Conjugated bili excreted into biliary system and intestine
Intestinal bili excreted as stool or becomes unconjugated and reenters bloodstream as
unconjugated bili through enterohepatic circulation
WHAT ARE THE MAIN CAUSES OF HYPERBILIRUBINEMIA IN THE NEONATE?
Increase hemolysis
Decreased hepatic uptake of unconjugated bili from circulation
Delayed maturation or inhibition of conjugating mechanism in liver
Increased enterohepatic circulation of unconjugated bili

WHAT TYPE OF BILI PUTS A NEONATE AT RISK FOR ACUTE ENCEPHALOPATHY

AND WHY?
Unconjugated only as is lipid soluble and passes the blood brain barrier, conjugated is water
soluble and cannot pass the BBB
WHAT ARE RISK FACTORS FOR SEVERE HYPERBILIRUBINEMIA?
Jaundice in the 1st 24 hr of life
Visible jaundice before hosp d/c
Fetal-maternal blood type incompatibility
Prematurity
Exclusive breastfeeding
Significant weight loss
Maternal age > 25
Male
Delayed meconium
Excessive birth trauma
LGA (in late preterms)
G6PD and breast feeding (in late peterms)
WHAT IS PHYSIOLOGIC JAUNDICE?
Transient elevation in bili that self resolves in 7-10 days
Peaks at day 3-5
WHAT ARE SIGNS OF PATHOLOGIC JAUNDICE?
Onset in 1st 24 hr
Rapidly rising bili level
anemia
Prolonged jaundice (> 3 weeks)
Conjugated bili > 20% of total bili
Temperature instability
Unwell appearing neonate
WHAT IS YOUR DDX FOR CONJUGATED (DIRECT) HYPERBILI OF THE
NEONATE?
Infection:
TORCH
Hepatitis
Sepsis
Anatomic:
Biliary atresia
Choledochal cyst
Cholestasis
Mass lesion
Alagille syndrome (ateriohepatic dysplasia)

Metabolic:
Inborn errors of metabolism
CF
Alpha-1-antitrypsin deficiency
WHAT IS YOUR DDX OF UNCONJUGATED (INDIRECT) HYPERBILI OF THE
NEONATE?
Benign:
Physiologic
Breast milk jaundice
Hemolysis:
ABO incompatibility
Physiologic breakdown of birth trauma hematomas
IVH
Spherocytosis
G6PD
Pyruvte kinase deficiency
Sickle cell
Infection:
TORCH
Sepsis (esp UTI)
Obstructive:
Meconium ileus
Hirschsrungs
Duodenal atresia
Pyloric stenosis
Metabolic/genetic:
Galactosemia
Congenital hypothyroidism
Crigler-Najjar syndrome (absence or deficient UDPGT)
Gilberts
WHY CAN BREASTFEEDING LEAD TO JAUNDICE?
Caloric derivation and dehydration from delayed milk production (takes 2-5 days before
maternal milk production up to normal) leading to increased enterohepatic circulation
Inhibition of hepatic B-UGT
WHAT PRESENTATIONS OF NEONATAL JAUNDICE REQUIRE A WORKUP?
Onset in the 1st 24 hr of life
Unwell appearing neonate (lethargy, irritability, poor feeding, temperature instability, vomiting)
> 20% TSB is conjugated
Onset after 7-10 d of life

Associated anemia
Persists after 3 weeks (exclude exclusively breast fed neonates without any of above and who
appear well)
WHAT ARE 3 TREATMENT MODALITIES USED TO TREAT POTENTIALLY
HARMFUL LEVELS OF HYPERBILIRUBINEMIA IN THE NEONATE?
Phototherapy:
Decreases bili level 15-25%
Blue light converts unconjugated bili to a water soluble compound that is excreted in urine and
stool
Ivig:
Used with fetal maternal ABO or Rh incompatibility
Exchange Transfusion:
Decreases bili level by ~ 50%
Indicated if: signs of intermediate to severe acute bilirubin encephalopathy, level as per AAP
guidelines, rising level despite phototherapy
REFERENCES
AAP Guidelines :Management of hyperbilirubinemia in the Newborn Infant 35 or more Weeks of
Gestation. Pediatrics. 2004;114(1): 297-316
Watchko, JF. Hyperbilirubinemia and Bilirubin toxicitiy in the Late Preterm Infant. Clin Perinat.
2006;33:839-852
Colletti, JE, et al. An Emergency Medicine Approach to Neonatal Hyperbilirubinemia. Emerg
Med Clin N AM. 2007;25:1117-1135