{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,15]],"date-time":"2026-08-15T10:34:24Z","timestamp":1786790064491,"version":"build-2736575974"},"reference-count":31,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T00:00:00Z","timestamp":1745366400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Cystic fibrosis hepato\u2010biliary involvement (CFHBI) is a common comorbidity in patients with CF and is associated with increased morbidity and mortality. The effect of the new and highly potent CF transmembrane conductance regulator modulator therapy, elexacaftor\u2013tezacaftor\u2013ivacaftor (ETI), on CFHBI, is still unclear. This study aimed to investigate the impact of ETI on liver stiffness in children with CF, as measured using two\u2010dimensional (2D) shear wave elastography (SWE).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Twenty\u2010one children with CF were included in this retrospective study at the CF centre, Sk\u00e5ne University Hospital, Lund, Sweden. Twelve children of our cohort had CFHBI; none had advanced CF liver disease. 2D SWE data from annual assessments, clinical data and liver enzymes were analysed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We found a significant reduction in liver stiffness after starting treatment with ETI in the total cohort. This reduction in liver stiffness could even be seen in children with CFHBI. Liver enzymes were within the normal range in both pre\u2010 and post\u2010ETI therapy in the total cohort. In children with CFHBI, a decline in aspartate aminotransferase activity was observed after ETI was initiated. Lung function and lung clearance index improved significantly after ETI treatment commenced.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>ETI treatment could positively affect CFHBI in children with CF, as demonstrated by reduced liver stiffness during treatment.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jpn3.70050","type":"journal-article","created":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T03:09:58Z","timestamp":1745377798000},"page":"74-81","source":"Crossref","is-referenced-by-count":3,"title":["The effect of elexacaftor\u2013tezacaftor\u2013ivacaftor on liver stiffness in children with cystic fibrosis"],"prefix":"10.1002","volume":"81","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-3319-611X","authenticated-orcid":false,"given":"Stefanie","family":"Diemer","sequence":"first","affiliation":[{"name":"Department of Paediatrics Sk\u00e5ne University Hospital Lund Sweden"},{"name":"Wallenberg Centre for Molecular Medicine Lund Sweden"},{"name":"Department of Clinical Science Lund, Section III, Infection Medicine Lund University Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helga","family":"Elidottir","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences Lund, Section V, Paediatrics Lund University Lund Sweden"},{"name":"Children's Hospital Landspitali \u2013 The National University Hospital of Iceland Reykjavik Iceland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erik A.","family":"Eklund","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Sk\u00e5ne University Hospital Lund Sweden"},{"name":"Department of Clinical Sciences Lund, Section V, Paediatrics Lund University Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa I.","family":"P\u00e5hlman","sequence":"additional","affiliation":[{"name":"Wallenberg Centre for Molecular Medicine Lund Sweden"},{"name":"Department of Clinical Science Lund, Section III, Infection Medicine Lund University Lund Sweden"},{"name":"Division of Infectious Diseases Sk\u00e5ne University Hospital Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Hansen","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Sk\u00e5ne University Hospital Lund Sweden"},{"name":"Department of Clinical Sciences Lund, Section V, Paediatrics Lund University Lund 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