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儿童和青少年威尔逊氏病(作者译)

[Wilson's liver disease in children and adolescents (author's transl)].

作者信息

Feist D, Wesch H

出版信息

Leber Magen Darm. 1979 Sep;9(5):235-9.

PMID:522594
Abstract

Hepatic symptoms are usually the first in Wilson's disease of children and adolescents, while neurologic symptoms and the corneal ring are still missing. Liver lesions due to copper accumulation may develop throughout years without clinical symptoms or biochemical abnormalities. Hemolytic jaundice or gastrointestinal bleeding are the presenting symptoms in some cases. In spite of being a rare syndrom Wilson's disease ought to be considered after hepatitis B or autoimmune liver disease have been excluded as causes of juvenile cirrhosis of the liver. If life-long treatment with D-penicillamin is started in an early stage of Wilson's disease, prognosis is rather good.

摘要

在儿童和青少年威尔逊病中,肝脏症状通常是首发症状,而此时神经症状和角膜环仍未出现。由于铜蓄积导致的肝脏损害可能在数年内逐渐发展,而无临床症状或生化异常。在某些情况下,溶血黄疸或胃肠道出血是首发症状。尽管威尔逊病是一种罕见综合征,但在排除乙型肝炎或自身免疫性肝病作为青少年肝硬化病因之后,仍应考虑该病。如果在威尔逊病早期开始用青霉胺进行终身治疗,预后相当良好。

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