Etiology, pathophysiology and management of reye’s syndrome

Abstract

Reye’s syndrome is defined as a fatal biphasic disorder that clinically described by preceding viral illness, protracted vomiting from one to two days before the onset of encephalopathy and liver dysfunction. Reye’s syndrome can be characterized as a constellation of delirium, fever, convulsions, vomiting, respiratory collapses, stupor, seizures, or coma typically following an earlier viral illness. Encephalopathy can be frequently progresses rapidly from lethargy to coma within twenty four to forty eight hrs. Both universal mitochondrial injury and triglyceride accumulations are the cornerstone etiology of Reye’s syndrome. Accumulation of high concentration of ammonia leads to encephalopathy and anicteric hepatitis with three times rise in liver enzymes. A frequent pathophysiological mechanism of Reye’s syndrome is induction of the mitochondrial permeability transition. The syndrome is correlated with a high mortality rate and the treatment is symptomatic including intensive care management with correction of metabolic abnormalities especially of hypotension, hypo glycaemia and acidosis, control of convulsions, and monitoring of intracranial hypertension due to cerebral edema. Agents to decrease serum ammonia concentrations are also usually used, the most frequent being are neomycin sulfate or lactulose. Anti-emetic such as ondansetron should be given to inhibit vomiting and potential aspiration.