{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T23:12:50Z","timestamp":1773011570498,"version":"3.50.1"},"reference-count":34,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,2,18]],"date-time":"2026-02-18T00:00:00Z","timestamp":1771372800000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T00:00:00Z","timestamp":1769904000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["005206Q123"],"award-info":[{"award-number":["005206Q123"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["#005583Q123"],"award-info":[{"award-number":["#005583Q123"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["LEUNG23A0"],"award-info":[{"award-number":["LEUNG23A0"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["HELTSH20K0"],"award-info":[{"award-number":["HELTSH20K0"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005564","name":"Gilead Sciences","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005564","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Elexacaftor\u2010tezacaftor\u2010ivacaftor (ETI) has significantly improved lung function and growth in people with cystic fibrosis (pwCF). However, its impact on cystic fibrosis hepatobiliary involvement (CFHBI) remains unclear. This study aims to investigate the impact of ETI on biochemical surrogates of CFHBI using a large population database.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      This 1:1 retrospective cohort study utilized TriNetX, a de\u2010identified multicenter database, and included people aged 6\u201321 years with ICD\u201010 diagnostic codes for cystic fibrosis who were prescribed pancreatic enzyme replacement therapy. ETI group (pwCF on ETI seen from 1\/1\/2020 to 1\/1\/2024) and non\u2010ETI group (pwCF not on ETI seen from 1\/1\/2014 to 1\/1\/2018) were analyzed. Groups were matched using propensity scores, and statistical analyses were performed on TriNetX (significance set at\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.05).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      There were 533 children with CF (CwCF) aged 6\u201312 years and 1177 adolescents and young adults with CF aged 13\u201321 years in each cohort. In CwCF, the ETI group had significantly lower ALT (33\u2009\u00b1\u200944\u2009U\/L vs. 45\u2009\u00b1\u200957\u2009U\/L,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.003) and GGT levels (18\u2009\u00b1\u200920\u2009U\/L vs. 28\u2009\u00b1\u200951\u2009U\/L,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.004), as well as improved albumin and PT\/INR compared to non\u2010ETI group. In pwCF (13\u201321), the ETI group had significantly lower GGT (21\u2009\u00b1\u200926\u2009U\/L vs. 32\u2009\u00b1\u200963\u2009U\/L,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.002), and improved albumin and PT compared to non\u2010ETI group; however, indirect bilirubin was higher (0.7\u2009\u00b1\u20090.5\u2009mg\/dL vs. 0.3\u2009\u00b1\u20090.4\u2009mg\/dL,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Our findings suggest that earlier ETI use in children with CF is associated with improved liver enzymes, offering reassurance against widespread drug\u2010induced liver injury. Long\u2010term follow\u2010up including imaging and biopsy when available, pre\u2010 and post\u2010ETI, is needed to better assess the impact of ETI on CFHBI and advanced CF liver disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71522","type":"journal-article","created":{"date-parts":[[2026,2,19]],"date-time":"2026-02-19T07:51:51Z","timestamp":1771487511000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The Impact of Elexacaftor\u2010Tezacaftor\u2010Ivacaftor on Biochemical Surrogates of Cystic Fibrosis Hepatobiliary Involvement: A Retrospective Cohort Study"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-5990-9424","authenticated-orcid":false,"given":"Sasha\u2010Jane","family":"Abi\u2010Aad","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition University of South Florida Morsani College of Medicine Tampa Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3818-8283","authenticated-orcid":false,"given":"Athanasios","family":"Tsalatsanis","sequence":"additional","affiliation":[{"name":"Research Methodology and Biostatistics Core University of South Florida Morsani College of Medicine Tampa Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-7645-4685","authenticated-orcid":false,"given":"Michael","family":"Narkewicz","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Digestive Health Institute Children's Hospital Colorado, School of Medicine University of Colorado Aurora Colorado USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel H.","family":"Leung","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition Texas 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