临床肝胆病杂志2017，Vol.33Issue(8)：1543-1547,5.DOI:10.3969/j.issn.1001-5256.2017.08.027
药物治疗儿童肝豆状核变性肝功能失代偿的效果观察
Effect of pharmacotherapy on decompensated liver function in children with hepatolenticular degeneration
李春霞 1龚敬宇 2张梅虹 2王建设3
作者信息
- 1. 延安大学附属医院感染病科,陕西延安716000
- 2. 上海复旦大学附属金山医院儿科,上海201500
- 3. 上海复旦大学附属儿科医院肝病中心,上海201102
- 折叠
摘要
Abstract
Objective To investigate the effect of pharmacotherapy on decompensated liver function in children with hepatolenticular degeneration.Methods Three children with "liver cirrhosis and decompensated liver function" as the onset of hepatolenticular degeneration who were admitted to Jinshan Hospital Affiliated to Fudan University from August 1,2015 to August 1,2016 were enrolled,and ATP7B gene sequencing was performed to make a confirmed diagnosis.Clinical outcome was recorded.Results Compound heterozygous mutation of the ATP78 gene was detected in the three children.Mter comprehensive medical treatment,all children had improvements in hypoproteinemia and coagulation function,and two children had a reduced Child-Pugh score.Conclusion Hepatolenticular degeneration should be considered for children with unexplained abnormal liver function and even liver cirrhosis,and early diagnosis and timely pharmacotherapy can reduce the need for liver transplantation.关键词
肝豆状核变性/肝硬化/药物疗法/儿童Key words
hepatolenticular degeneration/liver cirrhosis/drug therapy/child分类
医药卫生引用本文复制引用
李春霞,龚敬宇,张梅虹,王建设..药物治疗儿童肝豆状核变性肝功能失代偿的效果观察[J].临床肝胆病杂志,2017,33(8):1543-1547,5.
