Reye syndrome Laleh sardari fardmoghaddam Reye syndrome is
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Reye syndrome Laleh sardari fardmoghaddam
Reye syndrome is a rapidly progressive encephalopathy. [1] Symptoms may include vomiting, personality changes, confusion, seizures, and loss of consciousness. [2] Even though liver toxicity typically occurs, yellowish skin usually does not. [1] 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. [1] It usually begins shortly after recovery from a viral infection, such as influenza or chickenpox. [2] About 90% of cases are associated with aspirin (salicylate) use in children. [1] Inborn errors of metabolism are also a risk factor. [3] Changes on blood tests may include a high blood ammonia level, low blood sugar level, and prolonged prothrombin time. Often the liver is enlarged. [1] Prevention is typically by avoiding the use of aspirin in children. [2] When aspirin was withdrawn for use in children a decrease of more than 90% in rates of Reye syndrome was seen. [1] Early diagnosis improves outcomes. Treatment is supportive. [2] Mannitol may be used to help with the brain swelling. [1] The first detailed description of Reye syndrome was in 1963 by Douglas Reye. [4] Children are most commonly affected. It affects less than one in a million children a year. [1] The general recommendation to use aspirin in children was withdrawn because of Reye syndrome, with use of aspirin only recommended in Kawasaki disease.
Signs and symptoms Reye syndrome progresses through five stages
Stage I • Rash on palms of hands and feet • Persistent, heavy vomiting that is not relieved by not eating • Generalized lethargy • Confusion • Nightmares • No fever usually present[8] • Headaches
Stage II • • Stupor Hyperventilation Fatty liver (found by biopsy) Hyperactive reflexes
Stage III Continuation of Stage I and II symptoms Possible coma Possible cerebral edema Rarely, respiratory arrest
Stage IV Deepening coma • Dilated pupils with minimal response to light • Minimal but still present liver dysfunction •
Stage V Very rapid onset following stage IV Deep coma Seizures Multiple organ failure[9] Flaccidity Hyperammonemia (above 300 mg/d. L of blood) Death
Causes The precise mechanism by which Reye syndrome occurs is unknown. This serious condition is described as a "syndrome" rather than a disease as the clinical features that physicians use to diagnose it are quite broad.
Differential diagnosis Causes for similar symptoms include Various inborn metabolic disorders Viral encephalitis Drug overdose or poisoning Head trauma Liver failure due to other causes Meningitis Kidney failure Shaken baby syndrome
Prognosis Documented cases of Reye syndrome in adults are rare. The recovery of adults with the syndrome is generally complete, with liver and brain function returning to normal within two weeks of onset. In children, however, mild to severe permanent brain damage is possible, especially in infants. Over thirty percent of the cases reported in the United States from 1981 through 1997 resulted in fatality
Epidemiology Reye syndrome occurs almost exclusively in children. While a few adult cases have been reported over the years, these cases do not typically show permanent neural or liver damage. Unlike in the UK, the surveillance for Reye syndrome in the US is focused on patients under 18 years of age.
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