Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease

被引：24

作者：

Wen, Jessica ^{[1
,2
]}

机构：

[1] Childrens Hosp Philadelphia, Div Gastroenterol Hepatol & Nutr, Philadelphia, PA 19104 USA

[2] Univ Penn, Sch Med, Dept Pediat, Philadelphia, PA 19104 USA

来源：

CTS-CLINICAL AND TRANSLATIONAL SCIENCE | 2011年 / 4卷 / 06期

关键词：

liver disease; pediatrics; cirrhosis; RENIN-ANGIOTENSIN SYSTEM; TISSUE GROWTH-FACTOR; CLINICAL-EXPERIENCE; BILIARY DYSGENESIS; INTRACELLULAR CA2+; MAMMALIAN TARGET; EPITHELIAL-CELLS; LIVER FIBROSIS; DETECT CHANGES; PRIMARY CILIUM;

D O I：

10.1111/j.1752-8062.2011.00306.x

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

Autosomal recessive polycystic kidney disease (ARPKD) is a developmental disorder that mainly affects the kidneys and the biliary tract. Affected patients often have massively enlarged cystic kidneys as well as congenital hepatic fibrosis (CHF) characterized by dilated bile ducts and associated peribiliary fibrosis. This review will examine what is known about ARPKD-associated liver disease and will highlight areas of ongoing research into its pathogenesis and potential treatment. Clin Trans Sci 2011; Volume 4: 460465

引用

页码：460 / 465

页数：6

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