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KernicterusAka: Bilirubin Toxicity, Acute Bilirubin Encephalopathy

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  1. 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
  2. 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 (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
  3. Management
    1. See Nonphysiologic Neonatal Jaundice
  4. Prognosis
    1. Significant mortality and morbidity
  5. References
    1. Developmental follow-up of severe Hyperbilirubinemia
      1. Harris (2001) Pediatrics 107(5):1075

Kernicterus (C0022610)

Definition (MSH)A term used pathologically to describe BILIRUBIN staining of the BASAL GANGLIA; BRAIN STEM; and CEREBELLUM and clinically to describe a syndrome associated with HYPERBILIRUBINEMIA. Clinical features include athetosis, MUSCLE SPASTICITY or hypotonia, impaired vertical gaze, and DEAFNESS. Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the BLOOD-BRAIN BARRIER (e.g., SEPSIS). This condition occurs primarily in neonates (INFANT, NEWBORN), but may rarely occur in adults. (Menkes, Textbook of Child Neurology, 5th ed, p613)
Definition (CSP)brain disorder associated with high levels of bilrubin; clinical features include athetosis, muscle spasticity or hypotonia, impaired vertical gaze, and deafness; nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the blood-brain barrier; this condition occurs primarily in neonates with perinatal blood incompatibilities, but may rarely occur in adults.
ConceptsDisease or Syndrome (T047)
ICD9774.7
MSHD007647
EnglishBILIRUBIN ENCEPH, Bilirubin Encephalopathies, Bilirubin encephalopathy, ENCEPH BILIRUBIN, ENCEPH HYPERBILIRUBINEMIC, HYPERBILIRUBINEMIC ENCEPH, Hyperbilirubinemic Encephalopathies, Hyperbilirubinemic Encephalopathy, Kernicterus, Kernicterus of newborn, Nuclear jaundice
Spanishencefalopatia bilirrubinica, encefalopatia por bilirrubina, ictericia nuclear, kernicterus, kernicterus del recien nacido
Parent ConceptsBrain Diseases (C0006111), Hyperbilirubinemia (C0020433), Brain Diseases, Metabolic (C0006112), Erythroblastosis, Fetal (C0014761), Brain Diseases, Metabolic, Acquired (C0751744), Neonatal Jaundice (C0022353), Kernicterus (C0022610), Pigmentation (C0031911), Central nervous system dysfunction in newborn (C0270266), Duplicate concept (C1274013)
SourcesCOSTAR, CSP, CST, DXP, LCH, MSH, MTHICD9, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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