{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T22:51:04Z","timestamp":1783032664614,"version":"3.54.6"},"reference-count":30,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2025,11,24]],"date-time":"2025-11-24T00:00:00Z","timestamp":1763942400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators have been reported to improve lung function and reduce CF exacerbations. We aimed to investigate the efficacy of CFTR-modulators in CF-associated liver disease (CFLD) during long-term treatment. Longitudinal data were collected from genetically confirmed adult CF patients receiving CFTR-modulators. CFLD was diagnosed using conventional criteria combined with liver stiffness measurement (LSM). A total of 57 patients [56.1% male; median age at baseline (T0), 26 years (interquartile range [IQR], 23\u201335)] were included. Patients received lumacaftor\/ivacaftor and\/or elexacaftor\/tezacaftor\/ivacaftor for a median of 43 months (range, 15\u2013123) until last assessment (T2). The prevalence of CFLD decreased from 15 (26.3%) at T0 to 8 (14.0%) at T2 (p = 0.016), and no new cases of CFLD were observed. Median LSM decreased from 6.2 (IQR 4.9\u20138.0) to 5.0 kPa (IQR 4.1\u20136.2) in the overall cohort (p &lt; 0.001) and from 10.2 (IQR 6.8\u201313) to 6.2 kPa (IQR 5.0\u201312.4) in the CFLD subgroup (p = 0.025). Mild, transient fluctuations in liver enzymes occurred in 26.3% of patients. In conclusion, adults with CF receiving long-term CFTR modulators, showed improvement of liver disease assessed by ultrasonography and transient elastography. At the last assessment, half of the patients no longer met the criteria for CFLD.<\/jats:p>","DOI":"10.3390\/life15121794","type":"journal-article","created":{"date-parts":[[2025,11,24]],"date-time":"2025-11-24T10:24:17Z","timestamp":1763979857000},"page":"1794","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Improvement of Cystic Fibrosis-Associated Liver Disease in Adults on Long-Term Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators"],"prefix":"10.3390","volume":"15","author":[{"given":"Sofia","family":"Manioudaki","sequence":"first","affiliation":[{"name":"Intensive Care Unit, Sismanogleio General Hospital, 15126 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7926-3062","authenticated-orcid":false,"given":"Larisa","family":"Vasilieva","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Alexandra General Hospital, 11528 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleni","family":"Geladari","sequence":"additional","affiliation":[{"name":"3rd Department of Internal Medicine and Liver Outpatient Clinic, Evangelismos General Hospital, 10676 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iliana","family":"Mani","sequence":"additional","affiliation":[{"name":"2nd Department of Internal Medicine and Research Laboratory, Medical School, National and Kapodistrian University of Athens, Hippokration Hospital, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zoe","family":"Athanassa","sequence":"additional","affiliation":[{"name":"Intensive Care Unit, Sismanogleio General Hospital, 15126 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ioannis","family":"Elefsiniotis","sequence":"additional","affiliation":[{"name":"Hepatogastroenterology Unit, Academic Department of Internal Medicine, \u201cAgioi Anargyroi\u201d General and Oncology Hospital of Kifisia, National and Kapodistrian University of Athens, 14564 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emilia","family":"Hadziyannis","sequence":"additional","affiliation":[{"name":"2nd Department of Internal Medicine and Research Laboratory, Medical School, National and Kapodistrian University of Athens, Hippokration Hospital, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6725-3510","authenticated-orcid":false,"given":"Vasilios","family":"Sevastianos","sequence":"additional","affiliation":[{"name":"3rd Department of Internal Medicine and Liver Outpatient Clinic, Evangelismos General Hospital, 10676 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-4702-8411","authenticated-orcid":false,"given":"Aikaterini","family":"Oikonomou","sequence":"additional","affiliation":[{"name":"3rd Department of Internal Medicine, Sotiria Thoracic Diseases Hospital, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-6145-1661","authenticated-orcid":false,"given":"Andreas","family":"Theophilou","sequence":"additional","affiliation":[{"name":"Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filia","family":"Diamantea","sequence":"additional","affiliation":[{"name":"Adult Cystic Fibrosis Unit, Sismanoglio Hospital, 15126 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1991-7436","authenticated-orcid":false,"given":"Alexandra","family":"Alexopoulou","sequence":"additional","affiliation":[{"name":"2nd Department of Internal Medicine and Research Laboratory, Medical School, National and Kapodistrian University of Athens, Hippokration Hospital, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,11,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2519","DOI":"10.1016\/S0140-6736(16)00576-6","article-title":"Cystic fibrosis","volume":"388","author":"Elborn","year":"2016","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1016\/j.jcf.2008.03.009","article-title":"Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice","volume":"7","author":"Castellani","year":"2008","journal-title":"J. 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Care Med."}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/15\/12\/1794\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,24]],"date-time":"2025-11-24T10:47:31Z","timestamp":1763981251000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/15\/12\/1794"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,24]]},"references-count":30,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2025,12]]}},"alternative-id":["life15121794"],"URL":"https:\/\/doi.org\/10.3390\/life15121794","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,11,24]]}}}