Fatty infiltration in the liver in medium chain acyl CoA dehydrogenase deficiency

Fatty infiltration of the liver at postmortem examination has been recommended as a criterion for selection of infants who have died suddenly and unexpectedly for further biochemical investigation for disorders of fatty acid oxidation. We describe a boy with medium chain acyl CoA dehydrogenase defic...

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Bibliographic Details
Published in:Archives of disease in childhood 1991-06, Vol.66 (6), p.727-728
Main Authors: Losty, H C, Lee, P, Alfaham, M, Gray, O P, Leonard, J V
Format: Article
Language:English
Subjects:
Acyl-CoA Dehydrogenases - deficiency
Biological and medical sciences
Dietary Carbohydrates - therapeutic use
Epilepsy - etiology
Fatty Liver - pathology
Gastroenterology. Liver. Pancreas. Abdomen
Humans
Infant
Liver - pathology
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Male
Medical sciences
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Summary:Fatty infiltration of the liver at postmortem examination has been recommended as a criterion for selection of infants who have died suddenly and unexpectedly for further biochemical investigation for disorders of fatty acid oxidation. We describe a boy with medium chain acyl CoA dehydrogenase deficiency who died four months after diagnosis and in whom only minimal hepatic fatty infiltration was found.
ISSN:0003-9888
1468-2044
DOI:10.1136/adc.66.6.727