Acylcoenzyme A dehydrogenase deficiency in heart tissue from infants who died unexpectedly with fatty change in the liverBr Med J (Clin Res Ed) 1988; 296 doi: https://doi.org/10.1136/bmj.296.6614.11 (Published 02 January 1988) Cite this as: Br Med J (Clin Res Ed) 1988;296:11
- Fiona Allison,
- M J Bennett,
- S Variend,
- P C Engel
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.
Log in using your username and password
Log in through your institution
Register for a free trial to thebmj.com to receive unlimited access to all content on thebmj.com for 14 days.
Sign up for a free trial