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General Surgery – DOCTOR EB DEFINITION Reye's syndrome is a postinfectious triad consisting of encephalopathy, fatty liver degeneration, and transaminase elevation. EPIDEMIOLOGY & DEMOGRAPHICS • During the 1970s, 300 to 600 cases were being reported yearly in the U.S. • Since the mid-1980s, after the understanding that aspirin is associated with Reye's syndrome, the yearly count has fallen to <20 cases • Seasonal relation with influenza and varicella outbreaks • Age: rare in persons aged >18 yr; peak age (in the U.S.) is 6 to 8 yr • Case-fatality rate: 25% to 50% ETIOLOGY • Temporal association with influenza and varicella infection • Epidemiologic association with aspirin or other salicylate use to treat the viral infection • Possible association with aflatoxin and pesticides • Pathology • Liver: no inflammation; the striking finding is panlobular microvesicular hepatocyte infiltration on light microscopy and mitochondrial injury on electron microscopy • Brain: no inflammation but cerebral edema and anoxic degeneration • Pathogenesis: not fully understood but mitochondrial dysfunction is clearly center stage DIFFERENTIAL DIAGNOSIS • Inborn errors of metabolism (e.g., carnitine deficiency, ornithine transcarbamylase deficiency) • Salicylate or amiodarone intoxication • Jamaican vomiting sickness • Hepatic encephalopathy of any cause • Table E1-433 describes diseases that present a clinical or pathologic picture resembling Reye's syndrome. LABORATORY TESTS • Elevated transaminase (alanine aminotransferase and aspartate aminotransferase) • Elevated ammonia level • Occasional elevation of creatine phosphokinase, lactate dehydrogenase, and bilirubin and prolongation of prothrombin time • Occasional hypoglycemia (in patients <4 yr) • Cerebrospinal fluid is normal or contains <8 white blood cells per milliliter • Rarely a liver biopsy is indicated (in infants or in recurrent cases) TREATMENT • Supportive • Mannitol, glycerol, or hyperventilation for cerebral edema if present • Interferon-alfa (experimental) • Prevention Influenza vaccine Varicella vaccine Avoidance of aspirin in children, especially during influenza and varicella outbreaks Posted in General Surgery Leave a Comment on Reye's Syndrome