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Updated: Dec 13 2021

Reye Syndrome

  • 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
  • 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
  • imaging
    • Computed tomography (CT) of brain
      • diffuse brain swelling
  • Studies
    • Diagnostic testing
      • diagnostic approach
        • other causes of encephalopathy must be ruled out, including central nervous system infections
      • 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
  • Prognosis
    • Mortality < 20%
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