Last Updated: 2013-11-14
An acute encephalopathy with hepatic dysfunction stemming from mitochondrial damage.
Etiology remains unknown, but viral infections, exogenous toxins, drugs, and inborn errors of metabolism have been implicated. Aspirin is classically associated but data are lacking for a definitive cause-effect relationship.
Usually presents after a preceding viral infection with acute onset of profuse vomiting and altered mental status ranging from a personality change to coma.
Laboratory findings include elevated ammonia, ALT/AST, and prolonged PT, without an increase in bilirubin. Liver biopsy shows steatosis without significant inflammation.
Treatment includes intensive supportive care, correction of metabolic abnormalities, and control of intracranial pressure.
Prognosis is generally good, and liver function returns to normal. However, encephalopathy can result in permanent neurologic sequelae.
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