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

B. Taubman, Daniel Hale, R. I. Keeley

Research output: Contribution to journalArticle

44 Citations (Scopus)

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
StatePublished - Jan 1 1987

Fingerprint

Reye Syndrome
Brain Diseases
Coma
Hypoglycemic Agents
Hospital Emergency Service
Siblings
Fatty Acids
Medium chain acyl CoA dehydrogenase deficiency
Therapeutics

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health

Cite this

@article{17271e9ccb0d41c6aa761a94127d6fcd,
title = "Familial Reye-like syndrome: A presentation of medium-chain acyl-coenzyme A dehydrogenase deficiency",
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.",
author = "B. Taubman and Daniel Hale and Keeley, {R. I.}",
year = "1987",
month = "1",
day = "1",
language = "English (US)",
volume = "79",
pages = "383--385",
journal = "Pediatrics",
issn = "0031-4005",
publisher = "American Academy of Pediatrics",
number = "3",

}

Familial Reye-like syndrome : A presentation of medium-chain acyl-coenzyme A dehydrogenase deficiency. / Taubman, B.; Hale, Daniel; Keeley, R. I.

In: Pediatrics, Vol. 79, No. 3, 01.01.1987, p. 383-385.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Familial Reye-like syndrome

T2 - A presentation of medium-chain acyl-coenzyme A dehydrogenase deficiency

AU - Taubman, B.

AU - Hale, Daniel

AU - Keeley, R. I.

PY - 1987/1/1

Y1 - 1987/1/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=0023112673&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0023112673&partnerID=8YFLogxK

M3 - Article

C2 - 3822638

AN - SCOPUS:0023112673

VL - 79

SP - 383

EP - 385

JO - Pediatrics

JF - Pediatrics

SN - 0031-4005

IS - 3

ER -