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20年前瑞氏等在澳大利亚发现一新的小儿疾病,脑病伴肝脏受损。瑞氏综合征(RS)病人肝脏线粒体的损伤是一致性的,而其他组织的线粒体损伤则是局灶性的。故对RS的肝脏改变,特别是生物化学改变有较好的了解,可帮助洞察肝病的病因以及它的预后。材料与方法本研究是用从1977～1981年美国某2个儿童医院住院的RS(1～5期)病人中(活检或尸解)取得的肝组织,以光学和电子显微镜证实诊断。此外,设立了两个不同的对照组。即正常肝组18例,RS组20例,非瑞氏综合征其他肝病组12例。本研究选择的肝酶包括细胞溶酶,乳酸脱氢酶(LDH),葡萄糖6磷酸脱氨酶(GPDH),过氧化物酶体酶[过氧化氢酶(CATAL)]以及两种线粒体酶[谷氨酸脱氨酶(GDH),单胺氧化酶(MAO)。结果LDH在上述3组中全部平均值为769u。GDH在RS组比其他2组明显减少,有显著差别(P<0.05),特别是较正常组酶活性显著减少。MAO在RS组比其他2组明。显减少(P<0.05).
Two decades ago, Watson found a new pediatric disease in Australia with encephalopathy and liver damage. Damage to the liver mitochondria is consistent in patients with Reye’s syndrome (RS), while mitochondrial damage in other tissues is focal. Therefore, changes in the liver of RS, especially biochemical changes have a better understanding, can help to understand the etiology of liver disease and its prognosis. Materials and Methods This study was performed using optical and electron microscopy to confirm the presence of liver tissue from patients with RS (stage 1 to 5) hospitalizations (biopsy or autopsy) hospitalized in two US pediatric hospitals between 1977 and 1981. In addition, two different control groups were set up. 18 cases in normal liver group, 20 cases in RS group and 12 cases in other liver disease group without Reye’s syndrome. The liver enzymes selected in this study include cytolytic enzymes, lactate dehydrogenase (LDH), glucose 6 phosphate dehydrogenase (GPDH), peroxidase enzyme [catalase] and two mitochondrial enzymes [ Glutamate deaminase (GDH), monoamine oxidase (MAO). Results LDH in the above three groups all average 769u. GDH in RS group was significantly lower than the other two groups, there was a significant difference (P <0.05), especially compared with the normal group significantly decreased enzyme activity. MAO in the RS group than the other two groups. Significantly decreased (P <0.05).