Reye syndrome

Reye syndrome is a mysterious malfunction of the liver and brain, now believed to be caused by the interaction of aspirin with influenza-like viral infection or chickenpox.

Reye syndrome most commonly strikes children in the 4 to 12 year age range, with the peak incidence around age six. It was first noted to be epidemic in 1974, when over 400 cases were reported in the US. Then it most often appeared following epidemics of influenza type B or chickenpox. Researchers soon found a striking association of the use of aspirin-containing medicines following flu or chickenpox in Reye syndrome cases.

Reye syndrome usually occurs in a previously healthy child. A febrile illness, usually an upper respiratory tract infection (in 9 out of 10 of the cases), or chickenpox is followed by apparent recovery. Then suddenly, usually about 5 to 7 days after the beginning of the viral illness, there is sudden, severe and protracted vomiting. Delirium, combative behavior, and stupor may occur very soon after the onset of vomiting.

If the condition is unrecognized and untreated, symptoms in some cases may rapidly progress to seizures, coma, and death. The diagnosis is definitively made by blood tests for liver function and liver biopsy. There is liver enlargement with abnormal liver function but no jaundice. Effective control of the increased pressure within the brain (intracranial pressure) is a major factor in the outcome of the illness; the pressure within the skull must be continuously monitored and medication given to control it. Treatment also involves aggressive supportive care to correct the various metabolic abnormalities such as profoundly low blood sugar (hypoglycemia) and potential hemorrhage from blood clotting disorders.

Since 1987, there have not been more than 36 cases a year nationwide (and usually fewer than 20), due to aggressive recommendations to eliminate aspirin for fever control in children. Investigation of cases of Reye syndrome has parenthetically had a beneficial side effect of leading to the discovery of a number of rare genetic metabolic diseases that mimic Reye syndrome. It is my understanding that the mimics of Reye syndrome are now probably more common in total than the syndrome itself.

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