Liver disease in cystic fibrosis
Liver disease in cystic fibrosis
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DOI：
10.1097/mcp.0b013e3282f10a16
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发表时间：
2007-11-01
影响因子：
3.3
通讯作者：
Colombo, Carla
中科院分区：
文献类型：
--
作者：
Colombo, Carla
综述的目的本综述强调了囊性纤维化相关肝病的最新进展。囊性纤维化的广泛肝胆问题包括归因于潜在缺陷的特定改变以及医源性病变或反映肝脏外发生的疾病过程的影响。由胆道梗阻和进行性门静脉周围纤维化引起的局灶性胆汁性肝硬化是临床上最相关的问题，因为最初局灶性纤维化过程的扩展可能导致多小叶胆汁性肝硬化、门静脉高压症和最终肝衰竭。囊性纤维化相关肝脏疾病目前被归类为遗传性胆管病，是由于胆管细胞顶膜囊性纤维化跨膜调节因子缺乏或功能障碍所致。在自然史、风险因素、诊断方式和治疗选择的特征方面取得了重大进展。肝脏疾病是囊性纤维化的一种相对常见和早期的并发症。发病机制显然是多因素的，与环境和遗传因素的贡献。它对生活质量和生存的影响将在未来几年增加，其早期发现和治疗将成为越来越重要的问题。熊去氧胆酸是目前唯一可用的治疗方法，但正在评估新的治疗选择。
Purpose of review This review highlights recent developments in liver disease associated with cystic fibrosis. Recent findings The broad spectrum of hepatobiliary problems in cystic fibrosis includes specific alterations ascribable to the underlying defect as well as lesions of iatrogenic origin or that reflect the effects of a disease process occurring outside the liver. Focal biliary cirrhosis, resulting from biliary obstruction and progressive periportal fibrosis, is the most clinically relevant problem, because extension of the initially focal fibrogenic process may lead to multilobular biliary cirrhosis, portal hypertension and eventually liver failure. Cystic fibrosis associated liver disease is presently classified among genetic cholangiopathies and results from lack or dysfunction of the cystic fibrosis transmembrane regulator at the apical membrane of bile duct cells. Major advances have been achieved regarding characterization of natural history, risk factors, diagnostic modalities and treatment options. Summary Liver disease is a relatively frequent and early complication of cystic fibrosis. The pathogenesis is apparently multifactorial, with contributions from environmental and genetic determinants. Its impact on quality of life and survival will increase in future years, and its early detection and treatment will become increasingly important issues. Ursodeoxycholic acid is the only treatment currently available, but novel therapeutic options are being evaluated.