title: Cystic fibrosis related liver disease--another black box in hepatology.
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.
authors.affiliation: Division of Transplantation, Department of Surgery, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria. Katharina.Staufer@meduniwien.ac.at.
authors.forename: Katharina
authors.initials: K
authors.lastname: Staufer
authors.affiliation: Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria. Emina.Halilbasic@meduniwien.ac.at.
authors.forename: Emina
authors.initials: E
authors.lastname: Halilbasic
authors.affiliation: Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria. Michael.Trauner@meduniwien.ac.at.
authors.forename: Michael
authors.initials: M
authors.lastname: Trauner
authors.affiliation: Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria. Lili.Kazemi-Shirazi@meduniwien.ac.at.
authors.forename: Lili
authors.initials: L
authors.lastname: Kazemi-Shirazi
mesh_terms: D015415:Biomarkers / Q000097:blood; D002352:Carrier Proteins / Q000378:metabolism; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000209:etiology; D003550:Cystic Fibrosis / Q000150:complications* / Q000378:metabolism; D003937:Diagnosis, Differential; D006801:Humans; D008107:Liver Diseases / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D016031:Liver Transplantation; D008562:Membrane Glycoproteins / Q000378:metabolism; D065626:Non-alcoholic Fatty Liver Disease / Q000175:diagnosis / Q000209:etiology
chemical_list: D015415:Biomarkers; D002352:Carrier Proteins; D008562:Membrane Glycoproteins; C037643:bile acid binding proteins
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