Reye syndrome

Abstract

This chapter describes Reye syndrome, which is an acute noninflammatory encephalopathy with fatty degenerative liver failure. Decrease in aspirin use in children, in response to its association with Reye syndrome, and the identification of medications, toxins, and inborn errors of metabolism (IEMs) that present with Reye-like syndrome manifestations have made Reye syndrome exceedingly rare. While recognizing the rarity of Reye syndrome, the diagnosis should be considered in any child with vomiting, altered mental status, and classic laboratory findings, but must be a diagnosis of exclusion. Early recognition and treatment of Reye and Reye-like syndromes, including possible IEMs, is essential to prevent death and optimize the potential for recovery without neurologic impairment. Support of airway, breathing, circulation, minimizing metabolic demands, avoiding catabolism, promoting anabolic state, correction of metabolic derangements and coagulopathy, ammonia detoxification, and prevention/treatment of increased intracranial pressure (ICP) are the mainstays of treatment