Reye Syndrome

Reye syndrome is a rare, progressive encephalopathy. It is observed most commonly in children ages 4 to 12 shortly after recovery from acute viral illnesses such as influenza or varicella. Reye syndrome may result in cerebral edema, brain injury, and death. It can be confused with medium-chain acyl-CoA dehydrogenase deficiency (MCADD) because of similar symptoms that can include hyperammonemia, hypoglycemia, vomiting, and liver dysfunction (fatty liver); Reye syndrome usually is has an associated rash. Aspirin use during viral illness is known to be associated with Reye syndrome, but the syndrome can occur in the absence of aspirin use. It has been postulated that the combination of viral illness with salicylate exposure results in an inhibition of long chain fatty acid oxidation. The fatty acid oxidation disorders were initially identified as recurrent Reye-like syndromes with recurrent hypoglycemia, hypoketonemia, microvesicular liver steatosis, and hepatic encephalopathy. [Trauner: 1988] [Tein: 2015]

Management of children with Reye syndrome involves early diagnosis, decreasing brain swelling, reversing the metabolic injury to the liver, and preventing complications of lung injury and cardiac arrest. Some studies have shown that infusions of hypertonic IV glucose, even in late stages of the syndrome, may prevent progression of the syndrome.