{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T02:37:23Z","timestamp":1791167843997,"version":"4.1.0"},"reference-count":9,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2023,1,20]],"date-time":"2023-01-20T00:00:00Z","timestamp":1674172800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/ http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:p>The cystic fibrosis (CF) transmembrane conductance regulator corrector\/potentiator combinations lumacaftor\/ivacaftor and elexacaftor\/tezacaftor\/ivacaftor improve sweat chloride, pulmonary function, and nutrition. Yet it is unclear whether they may also impact the progression of liver fibrosis, which is a substantial source of morbidity and mortality for patients with CF. We conducted a retrospective, single-center analysis of children and adolescents with CF treated with lumacaftor\/ivacaftor and\/or elexacaftor\/tezacaftor\/ivacaftor therapy, focusing on alterations in liver function tests and fibrosis indices using previously-established thresholds that corresponded with increased liver elastography. In pairwise comparisons of before and during treatment timepoints, we found that those with CF-associated liver involvement experienced significant decreases in gamma-glutamyl transferase, aspartate aminotransferase-to-platelet index, and gamma-glutamyl transferase-to-platelet ratio while on lumacaftor\/ivacaftor. These differences were not observed in patients treated with elexacaftor\/tezacaftor\/ivacaftor, nor were they observed in patients without underlying CF-associated liver disease. These results provide the first evidence that lumacaftor\/ivacaftor may improve liver fibrosis in children and adolescents with CF and suggest it may be beneficial in the treatment of CF-associated liver disease.<\/jats:p>","DOI":"10.1097\/hc9.0000000000000010","type":"journal-article","created":{"date-parts":[[2023,1,20]],"date-time":"2023-01-20T14:00:29Z","timestamp":1674223229000},"page":"e0010-e0010","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":39,"title":["Effects of CFTR modulators on serum biomarkers of liver fibrosis in children with cystic fibrosis"],"prefix":"10.1097","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3324-5464","authenticated-orcid":false,"given":"Steven","family":"Levitte","sequence":"first","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Stanford University, Palo Alto, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4990-6279","authenticated-orcid":false,"given":"Yonathan","family":"Fuchs","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Stanford University, Palo Alto, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6985-9700","authenticated-orcid":false,"given":"Russell","family":"Wise","sequence":"additional","affiliation":[{"name":"Department of Pharmacy, Lucile Packard Children\u2019s Hospital Stanford, Palo Alto, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5352-7760","authenticated-orcid":false,"given":"Zachary M.","family":"Sellers","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Stanford University, Palo Alto, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2023,2]]},"reference":[{"key":"R1-20241015","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1056\/NEJMoa1409547","article-title":"Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR","volume":"373","author":"Wainwright","year":"2015","journal-title":"N Engl J Med"},{"key":"R2-20241015","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single Phe508del allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N Engl J Med"},{"key":"R3-20241015","doi-asserted-by":"crossref","first-page":"e0212779","DOI":"10.1371\/journal.pone.0212779","article-title":"The emerging burden of liver disease in cystic fibrosis patients: a UK nationwide study","volume":"14","author":"Toledano","year":"2019","journal-title":"PLoS 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