Reyes syndrome

What is Reyes syndrome? 

Reyes syndrome is a rare, acute noninflammatory encephalopathy that primarily affects children and adolescents.

A correlation between the disease and a preceding viral infection (especially influenza and varicella) treated with salicylates has been reported, although other toxic, metabolic, or hypoxic insults may play roles in the pathogenesis.

The clinical manifestations of Reyes syndrome include hyperammonemia, hypoglycemia, coagulopathy, and cerebral edema.

Treatment is supportive and includes administration of intravenous glucose to prevent hypoglycemia, and in severe cases, hyperventilation and intravenous mannitol to reduce intracranial pressure. 

Defects in fatty acid oxidation, such as medium-chain acyl coenzyme A dehydrogenase deficiency, can also present as a Reye-like syndrome and may be more common than Reyes syndrome, warranting a workup for inborn errors of metabolism in affected children.


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