先天性胆汁酸合成障碍与胆汁淤积性肝病

被引：18

作者：

方玲娟

王建设

机构：

[1] 复旦大学附属儿科医院

来源：

临床肝胆病杂志 | 2010年 / 26卷 / 06期

关键词：

胆汁淤积,肝内; 先天性胆汁酸合成障碍;

D O I：

暂无

中图分类号：

R575 [肝及胆疾病];

学科分类号：

100201 [内科学];

摘要：

胆汁淤积性肝病病因多样,先天性胆汁酸合成障碍(CBAS)就是其中之一。CBAS多属于常染色体隐性遗传,由胆汁酸合成过程中的酶缺陷所致。虽罕见,但由于CBAS病人多数早期可通过口服补充胆汁酸及脂溶性维生素等治疗获得良好疗效,因此早期明确诊断尤为重要。现对此病的病因、临床表现、诊断、治疗等作一综述。

引用

收藏

页码：585 / 588

页数：4

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