Fulminant hepatic failure without evidence of cirrhosis in a case of Wilson's disease.
Fulminant hepatic failure without evidence of cirrhosis in a case of Wilson's disease.
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威尔逊病病例中没有肝硬化证据的暴发性肝衰竭。
DOI：
10.2169/internalmedicine1962.28.80
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发表时间：
1989
期刊：
影响因子：
--
通讯作者：
Yoshiyuki Niho
中科院分区：
文献类型：
--
作者：
Katsuaki Enomoto;Hiromi Ishibashi;Katsumi Irie;Y. Okumura;Hideyuki Nomura;Masahiro Fukushima;Shoichi Inaba;Yoshiyuki Niho
我们治疗了一名患有威尔逊氏病的暴发性肝功能衰竭的 16 岁日本女孩。由于血清铜和铜蓝蛋白水平低且尿铜排泄量高，入院后立即诊断为威尔逊病。她的肝功能衰竭还伴随着一阵阵溶血危机。尽管给予了 D-青霉胺并反复进行血浆置换，她还是在四个月后死于肝功能衰竭。尸检时，肝脏表面光滑。肝脏的组织学显示大量坏死。只剩下少量分散的肝细胞，其中罗丹宁染色显示出铜。没有肝硬化的证据。此前报道的伴有暴发性肝衰竭的威尔逊病病例的肝脏均出现肝硬化。我们的病例表明，威尔逊氏病可能以真正的暴发性肝衰竭的形式发生，而没有先期的神经和肝病症状以及肝硬化的证据。
We treated a sixteen-year old Japanese girl with fulminant hepatic failure in Wilson's disease. The diagnosis of Wilson's disease was made immediately after the admission because of low serum copper and ceruloplasmin levels with high urinary copper excretion. Her hepatic failure was accompanied by bouts of hemolytic crisis. In spite of the administration of D-penicillamine and repeated plasmapheresis, she died of hepatic failure four months later. At autopsy, the surface of the liver was smooth. The histology of the liver showed massive necrosis. There were only a few remaining scattered hepatocytes, in which copper was revealed by Rhodanine staining. There was no evidence of cirrhosis. The livers of the previously reported cases of Wilson's disease accompanied by fulminant hepatic failure were all cirrhotic. Our case indicated that Wilson's disease could occur as true fulminant hepatic failure without preceeding neurological and hepatological signs and the evidence of cirrhosis.