Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

International Journal of Molecular Sciences

Katharina Staufer²  Emina Halilbasic¹  Michael Trauner¹ 

[1] Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria; E-Mails:;Division of Transplantation, Department of Surgery, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria

关键词: cystic fibrosis;    bile acid transporters;    gut liver axis;    non-invasive diagnostics;    cholestasis;   

DOI  :  10.3390/ijms150813529

来源: mdpi

【 摘 要 】

Due to improved medical care, life expectancy in patients with cystic fibrosis (CF) has veritably improved over the last decades. Importantly, cystic fibrosis related liver disease (CFLD) has become one of the leading causes of morbidity and mortality in CF patients. However, CFLD might be largely underdiagnosed and diagnostic criteria need to be refined. The underlying pathomechanisms are largely unknown, and treatment strategies with proven efficacy are lacking. This review focuses on current invasive and non-invasive diagnostic standards, the current knowledge on the pathophysiology of CFLD, treatment strategies, and possible future developments.

【 授权许可】

CC BY   
© 2014 by the authors; licensee MDPI, Basel, Switzerland.