June 1999
ATLANTA - Cases of Reye's syndrome in the United States has declined dramatically since 1980, according to a report from the Centers for Disease Control and Prevention (CDC).
Ermias D. Belay, MD, and colleagues examined data reported by local or state health departments or practicing physicians to CDC's National Reye's Syndrome Surveillance System. A total of 1,207 cases of Reye's syndrome in children were reported to the CDC from 1981 to 1997. After a high of 555 cases were documented in 1980, the number of cases declined rapidly, and since 1987 fewer than 37 cases have been reported each year. About 40% of all cases were in children younger than 5 years of age; more than 90% of all cases were in children younger than 15.
Slightly more (51.9%) patients were girls, and 92.6% were white. Blacks comprised 5%; Asian and Pacific Islanders were 2.1%. and 4% were American Indian or Alaskan Natives.
Most patients had been ill at least once during the three weeks before the onset of Reye's syndrome, and most appeared to have taken aspirin. Nearly one-third of the identified patients with Reye's syndrome died.
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Reye's syndrome is a severe neurological disorder that usually develops after certain viral infections, such as varicella and influenza, and occurs almost exclusively in children. The syndrome is characterized by encephalopathy and fatty degeneration of the liver. Reye's syndrome causes confusion, agitation and delirium, and can result in long-term neurologic complications, coma and death in as many as one-third of affected patients. The exact cause of Reye's syndrome is unknown, and the condition may sometimes be difficult to diagnose clinically.
While the marked decline in the incidence of Reye's syndrome represents an important public health achievement, the findings raise several practical considerations for physicians and public health officials, according to Belay. Most importantly, cases of Reye's syndrome continue to be reported in the United States.
"For every case of Reye's syndrome that occurs in the United States, we think there may be many other children who are receiving aspirin as treatment for chickenpox and influenza-like illnesses. Therefore, we need to continue educating parents to avoid the use of aspirin in children who have these illnesses," Belay said. There continues to be a risk for some children who require long-term aspirin therapy for illnesses such as juvenile rheumatoid arthritis and Kawasaki disease.
According to the CDC surveillance data, 14 of 361 patients with Reye's syndrome for whom data on aspirin use were available, had regularly taken salicylate-containing medications for these conditions. Some previous studies have documented an even higher rate among children with rheumatoid arthritis.
These children should be vaccinated against varicella and receive an annual influenza vaccine, as recommended by the Advisory Committee on Immunization Practices.
"Their caregivers should also be informed about the risks of long-term aspirin treatment and trained to recognize the early symptoms of Reye's syndrome so that aspirin can be withdrawn if Reye's syndrome is suspected," the researchers said in their study, which was published recently in the New England Journal of Medicine.
In addition, because the disease is now rare, infants and children who are thought to have it should also be evaluated for inborn metabolic disorders that can resemble Reye's syndrome, Belay said.
Although Reye's syndrome is rare today, 20 years ago it was more common and often was reported in groups of children during or after outbreaks of influenza or chickenpox, according to Lawrence B. Schonberger, MD, one of the coauthors of the CDC study.
"It was the work of several states, in collaboration with CDC, during the late 1970s and early 1980s that initially focused attention on the problem of Reye's syndrome and a possible connection with aspirin," he said.
Because of these studies, the CDC in 1980 began cautioning parents and physicians about a possible association between Reye's syndrome and the use of aspirin to treat children with chickenpox or influenza-like illnesses. The surgeon general issued a formal advisory about the use of salicylates (specifically aspirin and aspirin-containing products) in 1982, and the Food and Drug Administration required that warning labels be used for all aspirin products, beginning in 1986.
"The timely dissemination of preventive messages to parents and healthcare providers played a critical role in raising the public's awareness of this problem and ultimately in reducing the illness and death caused by Reye's syndrome," said Belay.
For more information:
- Belay ED, Bresee JS, Holman RC, et al. Reye's syndrome in the United States from 1981 through 1997. N Engl J Med. 1999;340:1377-82.
- Monto AS. The disappearance of Reye's syndrome - a public health triumph. N Engl J Med. 1999;340:1423-24.
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