Clinical Research

Acylcoenzyme A dehydrogenase deficiency in heart tissue from infants who died unexpectedly with fatty change in the liver

Br Med J (Clin Res Ed) 1988; 296 doi: http://dx.doi.org/10.1136/bmj.296.6614.11 (Published 02 January 1988) Cite this as: Br Med J (Clin Res Ed) 1988;296:11
  1. Fiona Allison,
  2. M J Bennett,
  3. S Variend,
  4. P C Engel

    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.

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