Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

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 u...

Descripción completa

Detalles Bibliográficos
Autores principales: Staufer, Katharina, Halilbasic, Emina, Trauner, Michael, Kazemi-Shirazi, Lili
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2014
Materias:
Review
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4159809/
https://www.ncbi.nlm.nih.gov/pubmed/25093717
http://dx.doi.org/10.3390/ijms150813529
_version_ 1782334283915984896
author Staufer, Katharina
Halilbasic, Emina
Trauner, Michael
Kazemi-Shirazi, Lili
author_facet Staufer, Katharina
Halilbasic, Emina
Trauner, Michael
Kazemi-Shirazi, Lili
author_sort Staufer, Katharina
collection PubMed
description 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.
format Online
Article
Text
id pubmed-4159809
institution National Center for Biotechnology Information
language English
publishDate 2014
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-41598092014-09-18 Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology Staufer, Katharina Halilbasic, Emina Trauner, Michael Kazemi-Shirazi, Lili Int J Mol Sci Review 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. MDPI 2014-08-04 /pmc/articles/PMC4159809/ /pubmed/25093717 http://dx.doi.org/10.3390/ijms150813529 Text en © 2014 by the authors; licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution license (http://creativecommons.org/licenses/by/3.0/).
spellingShingle Review
Staufer, Katharina
Halilbasic, Emina
Trauner, Michael
Kazemi-Shirazi, Lili
Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology
title Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology
title_full Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology
title_fullStr Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology
title_full_unstemmed Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology
title_short Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology
title_sort cystic fibrosis related liver disease—another black box in hepatology
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4159809/
https://www.ncbi.nlm.nih.gov/pubmed/25093717
http://dx.doi.org/10.3390/ijms150813529
work_keys_str_mv AT stauferkatharina cysticfibrosisrelatedliverdiseaseanotherblackboxinhepatology
AT halilbasicemina cysticfibrosisrelatedliverdiseaseanotherblackboxinhepatology
AT traunermichael cysticfibrosisrelatedliverdiseaseanotherblackboxinhepatology
AT kazemishirazilili cysticfibrosisrelatedliverdiseaseanotherblackboxinhepatology

Ejemplares similares