Staufer, Katharina (2020). Current Treatment Options for Cystic Fibrosis-Related Liver Disease. International journal of molecular sciences, 21(22) MDPI 10.3390/ijms21228586
|
Text
ijms-21-08586.pdf - Published Version Available under License Creative Commons: Attribution (CC-BY). Download (266kB) | Preview |
Cystic Fibrosis-related liver disease (CFLD) has become a leading cause of morbidity and mortality in patients with Cystic Fibrosis (CF), and affects children and adults. The understanding of the pathogenesis of CFLD is key in order to develop efficacious treatments. However, it remains complex, and has not been clarified to the last. The search for a drug might be additionally complicated due to the diverse clinical picture and lack of a unified definition of CFLD. Although ursodeoxycholic acid has been used for decades, its efficacy in CFLD is controversial, and the potential of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators and targeted gene therapy in CFLD needs to be defined in the near future. This review focuses on the current knowledge on treatment strategies for CFLD based on pathomechanistic viewpoints.
Item Type: |
Journal Article (Review Article) |
|---|---|
Division/Institute: |
04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Visceral Surgery and Medicine |
UniBE Contributor: |
Staufer, Katharina |
Subjects: |
600 Technology > 610 Medicine & health |
ISSN: |
1422-0067 |
Publisher: |
MDPI |
Language: |
English |
Submitter: |
Rahel Fuhrer |
Date Deposited: |
23 Dec 2020 12:19 |
Last Modified: |
05 Dec 2022 15:43 |
Publisher DOI: |
10.3390/ijms21228586 |
PubMed ID: |
33202578 |
Uncontrolled Keywords: |
CFTR modulators cystic fibrosis-related liver disease liver transplantation ursodeoxycholic acid |
BORIS DOI: |
10.48350/150006 |
URI: |
https://boris.unibe.ch/id/eprint/150006 |
Actions (login required)
 |
Edit item |