Reye syndrome

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 with acute onset of profuse vomiting and altered mental status, ranging from a personality change to coma, in children recovering from a recent viral infection.

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.

Reye syndrome is an illness that meets all the following criteria:[1]Centers for Disease Control and Prevention. Reye syndrome: 1990 clinical case definition. 1990 [internet publication]. http://wwwn.cdc.gov/nndss/conditions/reye-syndrome/case-definition/1990 an acute, noninflammatory encephalopathy with either sterile CSF containing <9 WBC/mL or cerebral edema without inflammatory cell infiltrate; hepatic dysfunction documented by either a threefold elevation of serum transaminases and/or serum ammonia, or liver biopsy demonstrating fatty infiltration; there is no other diagnosis to account for the cerebral and hepatic derangement.