The cause of Reye's syndrome remains unknown; however, a link was found with the use of aspirin or other salicylates in children and adolescents who have a viral infection such as influenza, chicken pox or the common cold. The increased risk of contracting Reye's Syndrome is one of the main reasons that aspirin is not recommended for use in people under the age of 16.

The syndrome is named for Dr R. Douglas Reye, who, along with Dr George Johnson, published the first study of the syndrome in 1963, though the disease was first diagnosed as a unique illness in 1929. In 1980 studies in Ohio, Michigan and Arizona by Starko et al pointed to the use of aspirin during an upper respiratory tract infection and chicken pox as a potential indicators for the syndrome. A decrease of the use of aspirin in children during the 1980s resulted in a corresponding decrease in the number of cases of Reye's syndrome, dramatically in children under 10 (Arrowsmith et al 1987). However, it is worth noting that a decrease in the number of cases has also been observed in countries where children's aspirin is still in use. Further case studies have revealed 19 viruses in conjuction with salicylates, pesticides and aflatoxin as potential factors contributing to the disease.

Presentation

Symptoms and signs

Reye's syndrome progresses through two stages :

Stage I
- Persistent or continuous vomiting and/or nausea, except for children under two who may have diarrhea or hyperventilate.
- Signs of brain dysfunction appear : listlessness, loss of energy, lethargy, drowsiness
Stage II
- Personality changes : irritability and aggressive behavior
- Disorientation : confusion, irrational and combative behavior
- Delirium, convulsions and coma

Features

Early diagnosis is vital, otherwise death or severe brain damage may follow.

The disease causes hepatic steatosis with minimal inflammation and severe encephalopathy (with swelling of the brain). Jaundice is NOT usually present. The liver may become slightly enlarged and firm, and there is a change in the appearance of the kidneys. (Suchy, Frederick, Ed: J Liver Disease in Children. Mosby, St. Louis.1994. Chapter 36.

Prognosis

In adults who survive the acute illness the recovery is generally complete, with liver and brain function returning to normal within two weeks of the illness. In children however, permanent brain damage is possible, especially in infants, and ranges from mild to severe.

Differential diagnosis

Causes for similar symptoms include

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The fall of Reye syndrome - number of Reye syndrome cases in US has fallen from 555 in 1980 to fewer than 37 cases each year since 1987 - Brief Article
From FDA Consumer, 9/1/99

The fall of Reye syndrome ... The number of Reye syndrome cases, a serious disorder that can lead to long-term brain disorders or death, has fallen from 555 in 1980 to fewer than 37 cases each year since 1987, according to a recent report from the national Centers for Disease Control and Prevention. Researchers credit a word-of-mouth campaign spread to parents and doctors, cautioning them not to give aspirin to children who have chickenpox or influenza-like illnesses. In 1982, the U.S. surgeon general warned that using aspirin to treat viral infections could lead to Reye syndrome, and in 1986, FDA required warning labels on all aspirin products. Since then, acetaminophen has replaced aspirin in most medications for fever and aches in children. (New England Journal of Medicine, May 1999)

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