kernicterus; neonatal jaundice; neonatal hyperbilirubinemia

Etiology

• hemolysis
  • Rh disease
• polycythemia
• pyloric stenosis
• congenital hypothyroidism

Pathology

• inefficient uptake of bilirubin by immature hepatocytes predisposes to hyperbilirubinemia
• kernicterus is due to accumulation & deposition of bilirubin in the brain

Clinical manifestations

• usually develops in 1st week of life
• early
  • jaundice
  • absent startle reflex
  • poor feeding or sucking
  • lethargy
• mid-stage:
  • high-pitched cry
  • arched back with neck hyperextended backwards
  • bulging fontanel
  • seizures
• late
  • high-frequency hearing loss
  • mental retardation
  • muscle rigidity
  • movement disorder

Laboratory

• serum bilirubin > 20-25 mg/dL

Complications

• permanent brain damage
  • cerebral palsy
• hearing loss
• death

Management

• phototherapy with blue light (420-470 nm)
  • soft eye shields are used to prevent retinopathy
• exchange transfusions as indicated

More general terms

• metabolic brain disease
• neonatal jaundice