Reye's syndrome

Reye syndrome is a rapidly progressive encephalopathy. Symptoms may include vomiting, personality changes, confusion, seizures, and loss of consciousness. Even though liver toxicity typically occurs, jaundice usually does not. Death occurs in 20–40% of those affected and about a third of those who survive are left with a significant degree of brain damage. The cause of Reye syndrome is unknown. It usually begins shortly after recovery from a viral infection, such as influenza or chickenpox. About 90% of cases in children are associated with aspirin (salicylate) use. Inborn errors of metabolism are also a risk factor. Changes on blood tests may include a high blood ammonia level, low blood sugar level, and prolonged prothrombin time. Often the liver is enlarged. Prevention is typically by avoiding the use of aspirin in children. When aspirin was withdrawn for use in children a decrease of more than 90% in rates of Reye syndrome was seen. Early diagnosis improves outcomes. Treatment is supportive. Mannitol may be used to help with the brain swelling. The first detailed description of Reye syndrome was in 1963 by Douglas Reye, an Australian pathologist. Children are most commonly affected. It affects fewer than one in a million children a year. The general recommendation to use aspirin in children was withdrawn because of Reye syndrome, with use of aspirin only recommended in Kawasaki disease. Reye syndrome progresses through five stages: The cause of Reye syndrome is unknown. It usually begins shortly after recovery from a viral infection, such as influenza or chickenpox. About 90% of cases in children are associated with aspirin (salicylate) use. Inborn errors of metabolism are also a risk factor. The association with aspirin has been shown through epidemiological studies. The diagnosis of 'Reye Syndrome' greatly decreased in the 1980s, when genetic testing for inborn errors of metabolism was becoming available in developed countries. A retrospective study of 49 survivors of cases diagnosed as 'Reye's Syndrome' showed that the majority of the surviving patients had various metabolic disorders, particularly a fatty-acid oxidation disorder medium-chain acyl-CoA dehydrogenase deficiency. There is an association between taking aspirin for viral illnesses and the development of Reye syndrome, but no animal model of Reye syndrome has been developed in which aspirin causes the condition.