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Reye syndrome

Reye syndrome, or Reye-like system is a multisystem disease, affecting all organs of the body but especially the liver and the brain. Fat accumulates in the liver, and other organs, leading to the buildup of toxins in the blood. Pressure in the brain increases acutely, leading to symptoms such as vomiting, irritability, delirium, seizures, and coma. Outcome after Reye syndrome is related to the severity of the brain damage from brain swelling; children who have a very acute presentation that progresses rapidly are more likely to die than those with a slowly progressive illness with the slower development of brain edema.
Although we usually think of Reye syndrome as coming with, or after, a viral illness in a child treated with salicylates, several inborn errors of metabolism manifest as a Reye-like syndrome, sometimes as the first indication that a child has an inborn error of metabolism. Prior to the identification of MCADD and expanded newborn screening, symptomatic individuals often died at presentation and were given either the diagnosis of Sudden Infant Death Syndrome or Reye Syndrome. The Reye-like syndrome may also uncommonly present in adults, and can be associated with pregnancy.
Management of children with Reye syndrome involves early diagnosis, decreasing brain swelling, reversing the metabolic injury to the liver, and preventing complications of lung injury and cardiac arrest. There are no direct treatments for Reye syndrome, although some studies have shown that infusions of hypertonic IV glucose may be helpful, even in later stages of the syndrome. [Reye syndrome information from the NIH]

Authors

Author: Lynne M Kerr MD, PhD, 8/2008
Content Last Updated: 9/2008