Reye syndrome

Reye syndrome
Other namesReye's syndrome
Appearance of a liver from a child who died of Reye syndrome as seen with a microscope. Hepatocytes are pale-staining due to intracellular fat droplets.
Pronunciation
SpecialtyPediatrics
SymptomsVomiting, personality changes, confusion, seizures, loss of consciousness[1]
ComplicationsPersistent vegetative state, coma
CausesUnknown[2]
Risk factorsAspirin use in children, viral infection[1][2]
TreatmentSupportive care[1]
MedicationMannitol[2]
Prognosis1/3 long term disability[2][3]
FrequencyLess than one in a million children a year[2]
Deaths~30% chance of death[2][3]

Reye syndrome is a rapidly worsening brain disease.[2] Symptoms of Reye syndrome may include vomiting, personality changes, confusion, seizures, and loss of consciousness.[1] While liver toxicity typically occurs in the syndrome, jaundice usually does not.[2] Death occurs in 20–40% of those affected with Reye syndrome, and about a third of those who survive are left with a significant degree of brain damage.[2][3]

The cause of Reye syndrome is unknown.[2] It usually begins shortly after recovery from a viral infection, such as influenza or chickenpox.[1] About 90% of cases in children are associated with aspirin (salicylate) use.[2] Inborn errors of metabolism are also a risk factor.[3] The syndrome is associated with changes on blood tests such as a high blood ammonia level, low blood sugar level, and prolonged prothrombin time.[2] Often, the liver is enlarged in those who have the syndrome.[2]

Prevention is typically by avoiding the use of aspirin in children.[1] When aspirin was withdrawn for use in children in the US and UK in the 1980s, a decrease of more than 90% in rates of Reye syndrome was seen.[2] Early diagnosis of the syndrome improves outcomes.[1] Treatment is supportive;[1] mannitol may be used to help with the brain swelling.[2]

The first detailed description of Reye syndrome was in 1963 by Australian pathologist Douglas Reye.[4] The syndrome most commonly affects children.[2] It affects fewer than one in a million children a year.[2] The general recommendation to use aspirin in children was withdrawn because of Reye syndrome, with use only recommended in Kawasaki disease.[3]

  1. ^ a b c d e f g h "NINDS Reye's Syndrome Information Page". NINDS. September 25, 2009. Archived from the original on August 1, 2016. Retrieved August 8, 2016.
  2. ^ a b c d e f g h i j k l m n o p q Pugliese, A; Beltramo, T; Torre, D (October 2008). "Reye's and Reye's-like syndromes". Cell Biochemistry and Function. 26 (7): 741–6. doi:10.1002/cbf.1465. PMID 18711704. S2CID 22361194.
  3. ^ a b c d e Schrör, K (2007). "Aspirin and Reye syndrome: a review of the evidence". Paediatric Drugs. 9 (3): 195–204. doi:10.2165/00148581-200709030-00008. PMID 17523700. S2CID 58727745.
  4. ^ McMillan, Julia A.; Feigin, Ralph D.; DeAngelis, Catherine; Jones, M. Douglas (2006). Oski's Pediatrics: Principles & Practice. Philadelphia: Lippincott Williams & Wilkins. p. 2306. ISBN 9780781738941. Archived from the original on August 15, 2016.

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