Management of polycystic liver disease

被引：25

作者：

Everson G.T. ^{[1
]}

Taylor M.R.G. ^{[1
]}

机构：

[1] Division of Gastroenterology and Hepatology, University of Colorado School of Medicine, Denver, CO 80262

来源：

Current Gastroenterology Reports | 2005年 / 7卷 / 1期

基金：

美国国家卫生研究院;

关键词：

Autosomal Dominant Polycystic Kidney Disease; Cystic Disease; Hepatic Cyst; Autosomal Recessive Polycystic Kidney Disease; Polycystic Liver Disease;

D O I：

10.1007/s11894-005-0061-6

中图分类号：

学科分类号：

摘要：

The adult forms of polycystic liver disease are characterized by autosomal dominant inheritance and numerous hepatic cysts, with or without renal involvement. Mutations in two distinct genes predispose to renal and liver cysts (PKD1 and PKD2), and mutations in two different genes yield isolated liver cysts (PRKCSH and SEC63). Mutations at certain loci of PKD1 may predispose to more severe renal cystic disease or cerebral aneurysms. Risk factors for severe hepatic cystic disease include aging, female sex, pregnancy, use of exogenous female steroid hormones, degree of renal cystic disease, or severity of renal dysfunction (in patients with mutations in PKD1 or PKD2). Although liver failure or complications of advanced liver disease is rare, some patients develop massive hepatic cystic disease and become clinically symptomatic. There is no effective medical therapy. Treatment options include cyst aspiration and sclerosis, open or laparoscopic cyst fenestration, hepatic resection, and liver transplantation. © Current Science Inc. 2005.

引用

页码：19 / 25

页数：6

共 49 条

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