Howat A J, Bennett M J, Variend S, Shaw L, Engel P C
Br Med J (Clin Res Ed). 1985 Jun 15;290(6484):1771-3. doi: 10.1136/bmj.290.6484.1771.
Two hundred consecutive cases of the sudden infant death syndrome were reviewed for the presence of fat in the liver; 14 showed diffuse panlobular microvesicular fatty change indistinguishable from that found in Reye's syndrome. Samples of frozen liver were available in five of the 14 cases; histochemical analysis showed well preserved cytochrome oxidase and succinate dehydrogenase activity in all five, uncharacteristic of Reye's syndrome. Fatty acyl-coenzyme A dehydrogenase activity in the liver was assayed biochemically in two of the same five cases with severe hepatic fatty infiltration; both showed a defect in medium chain acyl-coenzyme A dehydrogenase activity using the substrate octanoyl-coenzyme A. Both cases also showed cerebral oedema in association with fatty infiltration of renal tubules, myocardium, and skeletal muscle, characteristic of Reye's syndrome. It is concluded that diffuse panlobular microvesicular fatty change of the liver in victims of the sudden infant death syndrome, although essentially non-specific, indicates that the state of mitochondrial enzymes should be investigated.
对200例连续的婴儿猝死综合征病例进行了肝脏脂肪检查；14例显示弥漫性小叶微泡性脂肪变性，与瑞氏综合征所见无法区分。14例中有5例有冷冻肝脏样本；组织化学分析显示，所有5例的细胞色素氧化酶和琥珀酸脱氢酶活性保存良好，这并非瑞氏综合征的特征。在同一5例有严重肝脏脂肪浸润的病例中，对其中2例进行了肝脏中脂肪酰基辅酶A脱氢酶活性的生化测定；使用底物辛酰基辅酶A时，两者均显示中链酰基辅酶A脱氢酶活性存在缺陷。这2例还伴有肾小管、心肌和骨骼肌脂肪浸润的脑水肿，这是瑞氏综合征的特征。得出的结论是，婴儿猝死综合征受害者肝脏的弥漫性小叶微泡性脂肪变性虽然本质上是非特异性的，但表明应研究线粒体酶的状态。
