意外死亡且肝脏有脂肪变性的婴儿心脏组织中的酰基辅酶A脱氢酶缺乏症
Acylcoenzyme A dehydrogenase deficiency in heart tissue from infants who died unexpectedly with fatty change in the liver.
作者信息
Allison F, Bennett M J, Variend S, Engel P C
机构信息
Children's Hospital, Sheffield.
出版信息
Br Med J (Clin Res Ed). 1988 Jan 2;296(6614):11-2. doi: 10.1136/bmj.296.6614.11.
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.
摘要
对意外死亡且尸检显示肝脏有脂肪变化的婴儿的心肌，使用天然电子受体分析其长链和中链酰基辅酶A脱氢酶活性。在所调查的7例病例中，有2例发现酰基辅酶A脱氢酶活性缺乏。1例缺乏中链酰基辅酶A脱氢酶活性，另1例缺乏长链酰基辅酶A脱氢酶活性。这些发现强调了对意外死亡婴儿进行脂肪酸氧化研究的重要性。
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