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rdf:type |
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lifeskim:mentions |
|
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pubmed:issue |
6
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pubmed:dateCreated |
1985-7-25
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|
pubmed:abstractText |
An increasing number of reports indicate that patients with some inherited metabolic diseases may have symptoms resembling those of Reye syndrome. We describe two siblings who developed a Reye-like syndrome at ages 16 and 18 months, respectively, after a viral illness and salicylate therapy. Both had fasting hypoglycemia and hypoketonemia. At the time of the acute episode and after ingestion of a medium-chain triglyceride load, one of them excreted large amounts of abnormal metabolites derived from the omega- and (omega-1)-oxidation of medium-chain fatty acids. Medium-chain acyl-CoA dehydrogenase activity was lower than 20% of control values in fibroblasts from both patients. This enzyme defect should be considered in children with a Reye-like syndrome with these distinctive manifestations.
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pubmed:language |
eng
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pubmed:journal |
|
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pubmed:citationSubset |
AIM
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pubmed:chemical |
|
|
pubmed:status |
MEDLINE
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|
pubmed:month |
Jun
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pubmed:issn |
0022-3476
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pubmed:author |
|
|
pubmed:issnType |
Print
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pubmed:volume |
106
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
918-21
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pubmed:dateRevised |
2004-11-17
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pubmed:meshHeading |
pubmed-meshheading:3998948-Acyl-CoA Dehydrogenase,
pubmed-meshheading:3998948-Acyl-CoA Dehydrogenases,
pubmed-meshheading:3998948-Blood Glucose,
pubmed-meshheading:3998948-Carnitine,
pubmed-meshheading:3998948-Fatty Acids, Nonesterified,
pubmed-meshheading:3998948-Female,
pubmed-meshheading:3998948-Fibroblasts,
pubmed-meshheading:3998948-Humans,
pubmed-meshheading:3998948-Infant,
pubmed-meshheading:3998948-Liver,
pubmed-meshheading:3998948-Male,
pubmed-meshheading:3998948-Octanoic Acids,
pubmed-meshheading:3998948-Reye Syndrome
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pubmed:year |
1985
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|
pubmed:articleTitle |
Medium-chain acyl-CoA dehydrogenase deficiency in two siblings with a Reye-like syndrome.
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pubmed:publicationType |
Journal Article,
Case Reports
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