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Kernicterus

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Kernicterus, Bilirubin Toxicity, Bilirubin Encephalopathy, Acute Bilirubin Encephalopathy, Chronic Bilirubin Encephalopathy

  • See Also
  1. Neonatal Jaundice
  2. Risk Score for Neonatal Hyperbilirubinemia
  3. Nonphysiologic Neonatal Jaundice
  4. Breast Feeding Problems for the Infant
  5. Phototherapy
  6. Exchange Transfusion in Newborns
  1. Acute Bilirubin Encephalopathy: 1 infant in 10,000
    1. Progresses to Kernicterus in 5% of infants
  2. Chronic Bilirubin Encephalopathy (Kernicterus): 1 infant in 100,000
  • Risk Factors
  1. See Nonphysiologic Neonatal Jaundice
  2. Severe Hyperbilirubinemia (>20 mg/dl)
    1. Hyperbilirubinemia alone does not predict Bilirubin Encephalopathy
    2. For healthy infants at term with Serum Bilirubin >30 mg/dl, only 5% develop Bilirubin Encephalopathy
    3. Gamaleldin (2011) Pediatrics 128(4): e925-31 [PubMed]
  3. Earlier Gestational age
  4. Hemolysis
  5. Sepsis
  • Pathophysiology
  1. Unconjugated Bilirubin (indirect) elevation
  2. Total Bilirubin > 25 to 30 mg/dl
  3. Bilirubin necroses Neurons
    1. Basal Ganglia
    2. Globus Pallidus
    3. Putamen
    4. Caudate nuclei
  • Symptoms and Signs
  1. Stage 1 : Acute Bilirubin Encephalopathy
    1. Moro Reflex diminished
    2. Tone diminished
    3. Lethargy
    4. Poor feeding
    5. Vomiting
    6. High pitched cry
  2. Stage 2 (High mortality in this stage)
    1. Opisthotonus
    2. Seizure
    3. Irritability
    4. Fever
    5. Rigidity
    6. Oculogyric Crisis
    7. Upward gaze paralyzed
  3. Stage 3
    1. Spasticity decreases at 1 week of age
  4. Stage 4: Kernicterus (Chronic Bilirubin Encephalopathy with Residual deficits)
    1. Spasticity
    2. Severe athetoid Cerebral Palsy
    3. High frequency Hearing Loss or Deafness
    4. Mild Mental Retardation
    5. Upward Gaze Paralysis
    6. Dental dysplasia
  • Management
  1. See Nonphysiologic Neonatal Jaundice
  • Prognosis
  1. Significant mortality and morbidity
  2. Developmental follow-up is indicated for severe Hyperbilirubinemia
  • References
  1. Harris (2001) Pediatrics 107(5):1075-80 [PubMed]
  2. Muchowski (2014) Am Fam Physician 89(11): 873-8 [PubMed]