Reyes Syndrome

Reyes syndrome is an entity of unknown etiology affecting children, in which an upper respiratory tract infection, chicken pox, and, rarely, gastroenteritis are followed by vomiting, convulsion, coma, hypoglycemia, elevated blood ammonia, and abnormal serum transaminase values. Individuals dying of the entity show fatty metamorphosis of the liver, with multiple small fatty cyto-plasmic vesicles in the hepatocytes, myocardial fibers, and tubular cells of the kidneys. These are extremely fine vesicles compared with the coarse deposit seen in alcoholic fatty metamorphosis of the liver. Reyes syndrome can be confused with inborn errors of metabolism with which it may share many of the same clinical characteristics. The only way to be absolutely sure of the diagnosis is to demonstrate specific mitochondrial changes in liver tissue.

An increased incidence of this syndrome was noted in children who had taken aspirin for flu-like illnesses or chicken pox. Because of this, the use of aspirin in the treatment of children was discontinued in the 1980s. This has led to the virtual disappearance of the entity. Thus, from 1980 to 1997, 1207 cases of Reyes Syndrome were reported with a peak incidence of 555 cases in 1980.57 Since 1994, no more than two cases per year have been reported.

Reye Syndrome Rash
Figure 3.13 Meningococcemia. (A and B) Blotchy erythematous rash with petechiae and purpura (continued).

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