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Snapshot
  • A 3-year-old previously healthy boy is rushed to the emergency room for sudden-onset vomiting and lethargy. He was born at full-term without complications and had been meeting all milestones. Two weeks ago, he had headaches, myalgias, and fevers and tested positive for influenza A virus. At that time, his parents tried multiple anti-pyretic medications, including ibuprofen, acetaminophen, and aspirin. On physical exam, he is dehydrated and has hepatomegaly. Labs show increased liver enzymes and ammonia. A head computed tomography (CT) shows diffuse cerebral edema. He is admitted to the intensive care unit (ICU) and started on steroids.
Introduction
  • Clinical definition
    • acute non-inflammatory hepatic encephalopathy resulting in fatty liver
  • Epidemiology
    • demographics
      • children
      • very rare
    • risk factors
      • viral infection treated with aspirin
        • varicella
        • influenza
        • gastroenteritis
        • rarely, vaccination with live viruses
  • Pathogenesis
    • aspirin metabolites inhibit mitochondrial enzymes
      • disrupts oxidative phosphorylation and fatty-acid beta-oxidation pathways
      • microvesicular fatty changes in the liver
  • Prognosis
    • mortality < 20%
Presentation
  • Symptoms
    • nausea and sudden-onset vomiting
    • diarrhea
    • lethargy
    • irritability
    • restlessness
    • delirium
    • seizures
    • coma
  • Physical exam
    • hepatomegaly
    • minimal jaundice
    • obtunded
    • in severe cases
      • permanent neurologic damage
      • fixed and dilated pupils
      • loss of oculovestibular reflexes
Studies
  • Diagnostic testing
    • diagnostic approach
      • other causes of encephalopathy must be ruled out, including central nervous system infections
    • imaging
      • computed tomography (CT) of brain
        • diffuse brain swelling
    • studies
      • serum labs
        • ↓ blood glucose
        • ↑ ammonia
        • ↑ hepatic enzymes
        • ↑ long-chain free fatty acid levels
      • liver biopsy
        • non-inflammatory fatty changes
      • cerebral spinal fluid analysis
        • normal
Differential
  • Inborn errors of metabolism, e.g., fatty-acid oxidation defects, urea cycle defects, etc.
    • distinguishing factors
      • typically presents in infancy
      • genetic testing
      • normal liver size
Treatment
  • Management approach
    • avoid use of aspirin in children, except when necessary, e.g., Kawasaki disease
    • treatment is largely supportive and neuro-protective
  • First-line
    • neuro-protection
      • modalities
        • mannitol
        • glycerol
        • dexamethasone
Complications
  • Permanent brain damage
  • Death
 

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