Mitchell R A, Arcinue E L, Partin J C, Partin J S, Ram M L, Chang C H, Smialek J, Sarnaik A
Pediatr Res. 1985 Jan;19(1):19-22. doi: 10.1203/00006450-198501000-00006.
Five enzymes were measured in 50 liver specimens (18 normal liver, 20 Reye liver, 12 diverse liver disorders other than Reye syndrome). The enzymes were: glutamic dehydrogenase (E.C. 1.4.1.3), monoamine oxidase (E.C. 1.4.3.4), lactate dehydrogenase (E.C. 1.1.1.27), D-glucose-6-phosphate dehydrogenase (E.C. 1.1.1.49), catalase (E.C. 1.11.1.6). The Reye syndrome group showed significant decreases in glutamic dehydrogenase (56%) and monoamine oxidase (70%) compared to normal control tissue and these changes were not characteristic of the non-Reye liver disorder group as a whole. Neither catalase nor lactate dehydrogenase appeared to be altered significantly in the Reye or in the abnormal control group compared with normal controls. Thus, only the prominent decreases in the mitochondrial enzyme activities appeared to be highly characteristic of Reye syndrome. Paradoxically, the means of the five hepatic enzymes and the admission levels of two serum enzymes indicative of liver damage (alanine and aspartate aminotransferase) were remarkably similar for both survivors and nonsurvivors of Reye syndrome.
在50份肝脏标本(18份正常肝脏、20份瑞氏综合征肝脏、12份除瑞氏综合征外的其他各种肝脏疾病)中检测了5种酶。这些酶分别是:谷氨酸脱氢酶(酶学委员会编号1.4.1.3)、单胺氧化酶(酶学委员会编号1.4.3.4)、乳酸脱氢酶(酶学委员会编号1.1.1.27)、D - 葡萄糖 - 6 - 磷酸脱氢酶(酶学委员会编号1.1.1.49)、过氧化氢酶(酶学委员会编号1.11.1.6)。与正常对照组织相比,瑞氏综合征组的谷氨酸脱氢酶(降低56%)和单胺氧化酶(降低70%)显著下降,且这些变化并非整个非瑞氏肝脏疾病组的特征。与正常对照组相比,过氧化氢酶和乳酸脱氢酶在瑞氏综合征组或异常对照组中似乎均未发生显著改变。因此,只有线粒体酶活性的显著降低似乎是瑞氏综合征的高度特征性表现。矛盾的是,瑞氏综合征幸存者和非幸存者的5种肝脏酶的平均值以及两种指示肝脏损伤的血清酶(丙氨酸和天冬氨酸转氨酶)的入院水平非常相似。