Familial Reye-like syndrome: A presentation of medium-chain acyl-coenzyme A dehydrogenase deficiency

B. Taubman, D. E. Hale, R. I. Keeley

Research output: Contribution to journalArticle

Abstract

A 20-month-old girl with a family history of two siblings who died of an encephalopathy diagnosed as Reye syndrome presented to an emergency room in hypoglycemic coma and was found to have medium-chain acyl-coenzyme A dehydrogenase deficiency. The salient clinical and biochemical features of this newly described inborn error of fatty acid metabolism are described and contrasted to those of classical Reye syndrome. Important clues that should lead the clinician to suspect this disorder, methods of diagnosis, and appropriate acute and long-term therapy are also discussed.

Original languageEnglish (US)
Pages (from-to)383-385
Number of pages3
JournalPediatrics
Volume79
Issue number3
Publication statusPublished - 1987
Externally publishedYes

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ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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