What is Reye syndrome?

Updated: Apr 02, 2018
  • Author: Debra L Weiner, MD, PhD; Chief Editor: Kirsten A Bechtel, MD  more...
  • Print
Answer

Reye syndrome is characterized by acute noninflammatory encephalopathy and fatty degenerative liver failure. The syndrome was first described in 1963 in Australia by RDK Reye and described a few months later in the United States by GM Johnson. [17, 14] Cases with identical manifestations were described as early as 1929. In the United States, Reye syndrome became a reportable disease in 1973. Peak incidence was reported in 1979-80. [7, 8, 13]

Reye syndrome typically occurs after a viral illness, particularly an upper respiratory tract infection, influenza, varicella, or gastroenteritis, and is associated with the use of aspirin during the illness. A dramatic decrease in the use of aspirin among children, in combination with the identification of medication reactions, toxins, and inborn errors of metabolism (IEMs) that present with Reye syndrome–like manifestations, have made the diagnosis of Reye syndrome exceedingly rare.

With the recognition that Reye syndrome is rare, this condition should be considered in the differential diagnosis in any child with vomiting and altered mental status and classic laboratory findings. A high index of suspicion is essential. Given that manifestations of Reye syndrome are not unique to Reye syndrome but also are seen in a growing list of conditions, and given that no test is specific for Reye syndrome, the diagnosis must be one of exclusion.

All children with manifestations suggestive of Reye syndrome should be tested for IEM. Early recognition and treatment of Reye and Reye-like syndromes, including presumptive treatment for possible IEM (See Inborn Errors of Metabolism) is essential to prevent death and optimize the likelihood of recovery without neurologic impairment.

Some have suggested the term Reye syndrome or Reye-like syndrome should be used to describe clinical manifestations of diseases states regardless of etiology, while causes still without a known etiology after diagnostic workup should be referred to as Reye disease.


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!