Neonatology Book

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Kernicterus

Aka: Kernicterus, Bilirubin Toxicity, Acute Bilirubin Encephalopathy
  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):1075-80

Kernicterus (C0022610)

Definition (NCI) A rare neurologic disorder occurring in infants with jaundice. It results from brain damage by existing high levels of unconjugated-indirect bilirubin.
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.
Concepts Disease or Syndrome (T047)
MSH D007647
ICD10 P57, P57.9
SnomedCT 230770008, 206479002, 74925009, 50143004, 157135006, 268880009
English Bilirubin Encephalopathies, Bilirubin Encephalopathy, Encephalopathies, Bilirubin, Encephalopathy, Bilirubin, Kernicterus, KERNICTERUS, NUCLEAR JAUNDICE, Kernicterus of newborn, NOS, Kernicterus of newborn NOS, Kernicterus, unspecified, ENCEPH BILIRUBIN, BILIRUBIN ENCEPH, HYPERBILIRUBINEMIC ENCEPH, ENCEPH HYPERBILIRUBINEMIC, bilirubin encephalopathy, kernicterus, kernicterus (diagnosis), kernicterus of newborn (diagnosis), kernicterus of newborn, Kernicterus (disorder), Jaundice nuclear, Encephalopathies, Hyperbilirubinemic, Encephalopathy, Hyperbilirubinemic, Hyperbilirubinemic Encephalopathies, Hyperbilirubinemic Encephalopathy, Kernicterus of newborn NOS (disorder), Kernicterus [Disease/Finding], Nuclear jaundice, Bilirubin encephalopathy, Kernicterus of newborn, Kernicterus (morphologic abnormality), Kernicterus of newborn (disorder), encephalopathy; hyperbilirubinemic, hyperbilirubinemic; encephalopathy, jaundice; kernicterus, kernicterus; jaundice, kernicterus; newborn
Dutch bilirubine encefalopatie, encefalopathie; hyperbilirubinemisch, geelzucht; kernicterus, hyperbilirubinemisch; encefalopathie, kernicterus; geelzucht, kernicterus; pasgeborene, Kernicterus, niet gespecificeerd, kernicterus, Bilirubine-encefalopathie, Hyperbilirubinemische encefalopathie, Kernicterus
German Bilirubinenzephalopathie, Kernikterus, nicht naeher bezeichnet, Kernikterus, Bilirubin-Enzephalopathie, Hyperbilirubinämische Enzephalophatie
Portuguese Encefalopatia por bilirrubina, Icterícia nuclear, Kernicterus, Encefalopatia Bilirrubínica, Encefalopatia Hiperbilirrubinêmica
Spanish Encefalopatía por exceso de bilirrubina, Ictericia nuclear, Kernicterus, encefalopatía bilirrubínica, encefalopatía por bilirrubina, ictericia nuclear (anomalía morfológica), ictericia nuclear, kernicterus del recién nacido (trastorno), kernicterus del recién nacido, SAI (trastorno), kernicterus del recién nacido, SAI, kernicterus del recién nacido, kernícterus, Encefalopatía Bilirrubínica, Encefalopatía Hiperbilirrubinémica, Encefalopatia Bilirrubinica, Encefalopatia Hiperbilirrubinemica
Swedish Kernikterus
Japanese ﾋﾞﾘﾙﾋﾞﾝﾉｳｼｮｳ, ｶｸｵｳﾀﾞﾝ, 核黄疸, ビリルビン脳症
Czech kernikterus, Jádrový ikterus, Encefalopatie bilirubinová, Kernikterus
Finnish Kernikterus
Russian KERNIKTERUS, ENTSEFALOPATIIA BILIRUBINOVAIA, BILIRUBINOVAIA ENTSEFALOPATIIA, ZHELTUKHA IADERNAIA, БИЛИРУБИНОВАЯ ЭНЦЕФАЛОПАТИЯ, ЖЕЛТУХА ЯДЕРНАЯ, КЕРНИКТЕРУС, ЭНЦЕФАЛОПАТИЯ БИЛИРУБИНОВАЯ
Italian Encefalopatia iperbilirubinemica, Encefalopatia da bilirubina, Ittero nucleare
Korean 핵황달, 상세불명의 핵황달
Polish Kernicterus, Żółtaczka jąder podkorowych mózgu
Hungarian Mag-icterus, Bilirubin encephalopathia, Magicterus
French Ictère nucléaire néonatal, Ictère nucléaire, Encéphalopathie bilirubinique, Encéphalopathie hyperbilirubinémique, Encéphalopathie par hyperbilirubinémie, Kernictère
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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