Cystic fibrosis-associated liver disease

被引：65
|
作者：
Herrmann, Ulrike [1 ]
Dockter, Gerd [2 ]
Lammert, Frank [1 ]
机构：
[1] Univ Saarland, Saarland Univ Hosp, Dept Med 2, D-66421 Homburg, Germany
[2] Univ Saarland, Saarland Univ Hosp, Dept Paediat, D-66421 Homburg, Germany
来源：
BEST PRACTICE & RESEARCH CLINICAL GASTROENTEROLOGY | 2010年 / 24卷 / 05期
关键词：
Biliary fibrosis; Cystic fibrosis transmembrane conductance regulator fibrosing cholangiopathy; Microgallbladder; Mucoviscidosis; Ursodeoxycholic acid; URSODEOXYCHOLIC ACID; BICARBONATE SECRETION; MANAGEMENT; DIAGNOSIS; ADULTS; ULTRASOUND; CONSENSUS; OUTCOMES; RISK; ATP;
D O I：
10.1016/j.bpg.2010.08.003
中图分类号：
R57 [消化系及腹部疾病];
学科分类号：
摘要：
Liver disease is increasingly common in cystic fibrosis (CF) As new therapeutic options emerge life expectancy increases and common hepatobiliary manifestations impact on quality of life and survival of CF patients Hepatobiliary abnormalities in CF vary in nature and range from defects attributable to the underlying CFTR gene defect to those related to systemic disease and malnutrition Today complications of liver disease represent the third most frequent cause of disease-related death in patients with CF Here we review molecular and clinical genetics of CF including genetic modifiers of CF-associated liver disease and provide practical recommendations for genetic testing diagnosis and treatment of hepatobiliary manifestations in CF (C) 2010 Elsevier Ltd All rights reserved
引用
收藏
页码：585 / 592
页数：8
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