Acylcoenzyme A dehydrogenase deficiency in heart tissue from infants who died unexpectedly with fatty change in the liver. Issue 6614 (2nd January 1988)

• Record Type: Journal Article
• Authors: Allison, Fiona; Bennett, M J; Variend, S; Engel, P C
• Is Part Of: BMJ. Volume 296:Issue 6614(1988)
• Page Start: 11
• Page End: 12
• Publication Date: 1988-01-02
• Subjects: Medicine -- Periodicals; Medicine -- Periodicals; Medicine; Periodicals; 610
• DOI: 10.1136/bmj.296.6614.11
• Languages: English
• ISSNs: 0007-1447

Abstract

Abstract : Heart muscle from infants who died unexpectedly and who showed fatty changes in the liver at necropsy was analysed for long chain and medium chain acylcoenzyme A dehydrogenase activities by using the natural electron acceptor. In two of the seven cases investigated a deficiency in acylcoenzyme A dehydrogenase activity was found. In one case the deficiency was in medium chain acylcoenzyme A dehydrogenase activity and in the other long chain acylcoenzyme A dehydrogenase activity. These findings emphasise the importance of investigating fatty acid oxidation in infants who have died unexpectedly.