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Int. J. Mol. Sci. 2014, 15(8), 13529-13549; doi:10.3390/ijms150813529

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

1
Division of Transplantation, Department of Surgery, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria
2
Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria
*
Author to whom correspondence should be addressed.
Received: 1 May 2014 / Revised: 10 July 2014 / Accepted: 16 July 2014 / Published: 4 August 2014
(This article belongs to the Collection Molecular Mechanisms of Human Liver Diseases)
View Full-Text   |   Download PDF [709 KB, uploaded 4 August 2014]

Abstract

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. View Full-Text
Keywords: cystic fibrosis; bile acid transporters; gut liver axis; non-invasive diagnostics; cholestasis cystic fibrosis; bile acid transporters; gut liver axis; non-invasive diagnostics; cholestasis
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

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MDPI and ACS Style

Staufer, K.; Halilbasic, E.; Trauner, M.; Kazemi-Shirazi, L. Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology. Int. J. Mol. Sci. 2014, 15, 13529-13549.

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