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Case 1
• 5 day old former term male infant born to a
23 y.o. G1P0->1 woman. Is exclusively
breastfeeding. Has total bilirubin of 25,
direct is 0.7. Both mother and baby are O+,
and coombs is negative.
• Phototherapy is started and baby is fed, 6
hours later the total bilirubin level is 19.
• Breastfeeding consult is done.
Physiologic Hyperbilirubinemia
• Increased production
– Short RBC lifespan
– Increased shunt bilirubin
• Decrease clearance
– Portal vein shunting via ductus venosus
• Decreased conjugation
– Decreased UDPGA synthesis
– Decreased UDPG transferase
• Increased enterohepatic circulation
– High concentration of bilirubin in
meconium
– Decreased bowel motility
Hyperbilirubinemia: Elevation in disease states
• Overproduction • Undersecretion
– Decreased conjugation
– Hemolysis
• Congenital
• Isoimmunization
– Impaired hepatic transport
• Genetic
• Congenital
– Sequestered Blood • Liver disease
– Polycythemia – Biliary obstruction
– Increased
Enterohepatic
• Mixed
Circulation – Infections
• Bowel obstruction • Congenital infections
• Bacterial sepsis
• Intestinal
hypomotility – Prematurity
– Infant of a Diabetic Mother
– Hypothyroidism
Bilirubin Neurotoxicity
• What is kernicterus?
– Yellow staining of the brain
– Neuronal necrosis microscopically
• Getting bilirubin from the blood to the brain
cell isn’t easy
• “The numbers” keep changing
• Bilirubin encephalopathy vs. minor CNS
deficits
Clinical Features of Kernicterus
• Acute
– Phase 1 (first 1-2 days): poor sucking, stupor,
hypotonia, seizures
– Phase 2 ( mid first week): hypertonia of extensors,
opisthotonus, retrocollis, fever
– Phase 3 (after first week): hypertonia
• Chronic
– First year: hypotonia, active DTRs, obligate tonic
neck refles, delayed motor skills
– After first year: movement disorders (choreoathetosis,
ballismus, tremor), upward gaze, sensorineural
hearing loss.
Vigintiphobia*: Fear of “20”
• Based on retrospective analysis of infants
with hemolytic Rh disease…in the dark
ages before intrauterine transfusion.
– Hsia, et al. NEJM 1952;247:668-71
– Mollison and Cutbush. Blood 1951:777-88
90
80
70
60
50
40
30
20
10
0
<5 6-15 16-30 >31
Serum Bilirubin
AAP Guidelines for
Hyperbilirubinemia - 2004
• AMERICAN ACADEMY OF PEDIATRICS
CLINICAL PRACTICE GUIDELINE
Subcommittee on Hyperbilirubinemia
Management of Hyperbilirubinemia in the
Newborn Infant 35 or More Weeks of
Gestation
PEDIATRICS Vol. 114 No. 1 July 2004
Prevention of Kernicterus:
Identifying infants at risk for Hyperbilirubinemia
• Prenatal maternal blood type and antibody screen
• Blood type and direct coombs on the baby
– Or type only babies of:
• Rh negative mothers (necessary for Rhogam eval)
• Other antibody screen positive
• O mothers (for ABO incompatibility), or hold cord blood 5-7
days for testing
• Good follow up and check bilirubin levels
Risk Factors Without Hemolysis*
• Jaundice in first 24 hours
• Visible jaundice before discharge
• Previous jaundiced sibling
• Gestation 35-38 weeks
• Exclusive breastfeeding
• East Asian Race
• Bruising/cephalohematoma
• Maternal age >25 years
• Male sex