Cystic fibrosis-related liver disease
MONDO:7770005A liver disorder that arises as a consequence of cystic fibrosis. Cystic fibrosis-related liver disease encompasses a spectrum of hepatobiliary abnormalities caused by CFTR dysfunction in cholangiocytes, including focal biliary cirrhosis, multilobular biliary cirrhosis, hepatic steatosis, and cholangiopathy. It is a significant cause of non-pulmonary morbidity and mortality in CF, affecting approximately 5-10% of CF patients.
Also known as: CF liver disease, CF-related liver disease, CFLD
3 clinical trials for this condition and its sub-types, 3 tagged with Cystic fibrosis-related liver disease itself.
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Triple therapy under the microscope: does it transform cystic fibrosis care?
Disease control OngoingResearchers are observing how elexacaftor/tezacaftor/ivacaftor triple combination therapy affects people with cystic fibrosis in routine care. The study follows two groups: children aged 6 to 11 and people aged 12 and older. Over seven years, they measure lung function, inflammat…
Phase 4 • Sponsor: Royal College of Surgeons, Ireland • Aim: Disease control
Last updated Sep 11, 2026 00:00 UTC
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Scientists hunt for genes behind liver disease in cystic fibrosis
Knowledge-focused OngoingThis study looks at genes, other than the CF gene, that might explain why some people with cystic fibrosis develop severe liver disease while others do not. Researchers are analyzing blood samples and medical records from 154 participants with CF who have serious liver problems. …
Sponsor: University of North Carolina, Chapel Hill • Aim: Knowledge-focused
Last updated Jun 27, 2026 07:51 UTC