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Reye Syndrome

Reye syndrome is a rare, progressive encephalopathy. It is observed most commonly in children ages 4 to 12 shortly after recovery from acute viral illnesses such as influenza or varicella. Reye syndrome may result in cerebral edema, brain injury, and death. It can be confused with medium-chain acyl-CoA dehydrogenase deficiency (MCADD) because of similar symptoms that can include hyperammonemia, hypoglycemia, vomiting, and liver dysfunction (fatty liver); Reye syndrome usually is has an associated rash. Aspirin use during viral illness is known to be associated with Reye syndrome, but the syndrome can occur in the absence of aspirin use. It has been postulated that the combination of viral illness with salicylate exposure results in an inhibition of long chain fatty acid oxidation. The fatty acid oxidation disorders were initially identified as recurrent Reye-like syndromes with recurrent hypoglycemia, hypoketonemia, microvesicular liver steatosis, and hepatic encephalopathy. [Trauner: 1988] [Tein: 2015]
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. Some studies have shown that infusions of hypertonic IV glucose, even in late stages of the syndrome, may prevent progression of the syndrome.

Resources

Information & Support

For Professionals

Medications Containing Aspirin (National Reye Syndrome Foundation)
One-page list of over-the-counter and prescription medications containing aspirin.

For Parents and Patients

Support

National Reye Syndrome Foundation
Provides emotional support and guidance for families, supports research, and conducts awareness programs related to Reye syndrome.

General

Reye Syndrome (NIH)
Basic information about Reye syndrome; National Institutes of Health.

Patient Education

Reye Syndrome Awareness Fact Sheet (National Reye Syndrome Foundation)
One-page fact sheet with information about possible causes and early signs of Reye syndrome.

Helpful Articles

PubMed search for Reye syndrome, last 6 years.

Tein I.
Impact of fatty acid oxidation disorders in child neurology: from Reye syndrome to Pandora's box.
Dev Med Child Neurol. 2015;57(4):304-6. PubMed abstract

Authors

Author: Lynne M Kerr, MD, PhD - 8/2008
Reviewing Authors: Laurie Smith, MD, PhD - 5/2016
Natario Couser, MD, MS - 5/2016
Content Last Updated: 5/2016

Page Bibliography

Tein I.
Impact of fatty acid oxidation disorders in child neurology: from Reye syndrome to Pandora's box.
Dev Med Child Neurol. 2015;57(4):304-6. PubMed abstract

Trauner DA, Horvath E, Davis LE.
Inhibition of fatty acid beta oxidation by influenza B virus and salicylic acid in mice: implications for Reye's syndrome.
Neurology. 1988;38(2):239-41. PubMed abstract