Chandra R K
Trans R Soc Trop Med Hyg. 1976;70(4):296-301. doi: 10.1016/0035-9203(76)90079-1.
Indian childhood cirrhosis is a significant cause of morbidity and mortality in young children in India. One hundred patients with ICC, 66 boys and 34 girls, were studied. Pedigree analysis yielded a segregation ratio of 0-2196, suggestive of an autosomal recessive inheritance. Serum alpha1-antitrypsin level was normal. Serum alpha-foetoprotein (AFP) concentration was increased in all the patients, parents and in some siblings. Serum immunoglobulins G, A, M, and D were elevated. Haemolytic complement and C3 were low. Electrophoretically altered complement components were detected in 36% of patients. There was an inverse relationship between C3 concentration and immunoconglutinin titre. Circulatingimmune complexes were detected in the sera of six out of ten patients who had significant proteinuria. Hepatitis B surface antigen (HBsAg) was present in the serum, ascitic fluid, saliva, urine and faeces of ICC patients more frequently than in controls. HBsAb was detected less often. Lymphocyte response to HBsAg was impaired. The first-degree relatives had a higher incidence of HBsAg and HBsAb than healthy controls. It is suggested that ICC occurs in infants with an inherited hepatocyte vulnerability and that one of the precipitating causes of liver cell necrosis is infection with hepatitis virus(es). The consequent immunologic epiphenomena contribute to progressive hepatic damage ending in death.
印度儿童肝硬化是印度幼儿发病和死亡的一个重要原因。对100例印度儿童肝硬化患者进行了研究，其中66例为男孩，34例为女孩。系谱分析得出的分离比为0 - 2196，提示为常染色体隐性遗传。血清α1 -抗胰蛋白酶水平正常。所有患者、其父母及部分兄弟姐妹的血清甲胎蛋白（AFP）浓度均升高。血清免疫球蛋白G、A、M和D均升高。溶血补体和C3降低。在36%的患者中检测到电泳改变的补体成分。C3浓度与免疫胶固素滴度呈负相关。在10例有明显蛋白尿的患者中，有6例患者的血清中检测到循环免疫复合物。与对照组相比，印度儿童肝硬化患者血清、腹水、唾液、尿液和粪便中乙肝表面抗原（HBsAg）的出现频率更高。乙肝表面抗体（HBsAb）的检测频率较低。淋巴细胞对HBsAg的反应受损。一级亲属中HBsAg和HBsAb的发生率高于健康对照组。提示印度儿童肝硬化发生于具有遗传性肝细胞易损性的婴儿，肝细胞坏死的诱发原因之一是感染肝炎病毒。随之出现的免疫现象促使肝脏进行性损害直至死亡。
