Home   About the Journal  Editorial Board  Online Journal   Publishing Ethics   Contact Us  Chinese Version
You are here:Home => Online Journal => 6期 => 专论 =>
Congenital bile acid synthesis defect and cholestatic liver disease
文章发布日期:2010年10月19日  来源:  作者:方玲娟,王建设  点击次数:7036次  下载次数:1561次
Adjust font size:Small regular big

【Abstract】:The etiologies of cholestatic liver disease are various and complicated. Congenital bile acid synthesis defect (CBAS) is about 1%-2% of children’s cholestasis. CBAS usually occured by autosomal recessive inheritance, due to various enzymes defect in bile acid synthesis. Although very rare, CBAS are treatable, usually by replacement of the deficient primary bile acids. Therefore, early diagnosis and treatment are very important for patients with CBAS. This paper summarized the etiology, clinical manifestations, diagnosis, treatment and so on.
【Key words】:cholestasis, intrahepatic; congenital bile acid synthesis defect

Mail address: Editorial Office of Journal of Clinical Hepatology, 519 Dongminzhu Street, Changchun, Jilin, China Postal code: 130061 Tel:+86-431-88782542/3542
Journal of Clinical Hepatology Copyright © 2009 - 2013 Lcgdbzz.org. All Rights Reserv 吉ICP备10000617号