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自1912年报告此病以来,已有很多报导。有的以脑病变为主,有的以肝病变为主,故其临床症状和病程亦不一致。特别是儿科患者与典型病例多不相同,著者观察9例,叙述如下。例1 5岁7个月男孩。主诉为黄疸、腹水和肝脾肿大。其兄5岁时患黄疸和腹水,病后1个月死亡。患儿5岁6个月时出现黄疸、发热和肝脾肿大,以后黄疸及腹水加重,下肢浮肿。无锥体外系症状,眼科检查有角膜色素环。病后1个月死于肝昏迷。剖检证实有盱硬化及食管静脉曲张。
Much has been reported since the disease was reported in 1912. Some of the main brain lesions, and some liver disease-based, so the clinical symptoms and course of disease is also inconsistent. In particular, pediatric patients and typical cases are not the same, the author observed 9 cases, described below. Example 1 5 years old 7 months boy. The main complaint was jaundice, ascites and hepatosplenomegaly. His brother suffering from jaundice and ascites at 5 years of age, 1 month after the illness died. Children aged 5 and 6 months when jaundice, fever and hepatosplenomegaly, jaundice and ascites after exacerbations, lower extremity edema. No extrapyramidal symptoms, eye examination corneal pigment ring. 1 month after illness died of hepatic coma. Autopsy confirmed 盱 sclerosis and esophageal varices.