{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T02:16:16Z","timestamp":1787019376156,"version":"build-2736575974"},"reference-count":35,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["SAGE Open Medicine"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:sec>\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>This study aimed to conduct a systematic review and meta-analysis of randomized controlled trials to evaluate the effects of elexacaftor\u2013tezacaftor\u2013ivacaftor (ELX-TEZ-IVA) on patients with cystic fibrosis (CF).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>A systematic search was performed in PubMed, Embase, and the Cochrane Library from inception to August 1, 2022. Meta-analysis was conducted using Review Manager 5.3 software.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>Six studies comprising seven reports involving a total of 1125 CF patients were included. The meta-analyses indicated that ELX-TEZ-IVA significantly improved the percentage predicted forced expiratory volume in 1\u2009s (ppFEV1) by 10.29% (95% confidence interval (CI) (6.44, 14.14), p\u2009&lt;\u20090.00001) and the CF questionnaire-revised respiratory domain (CFQ-R RD) by 14.59\u2009points (95% CI (9.25, 19.94), p\u2009&lt;\u20090.00001) compared to placebo, ivacaftor (IVA), or tezacaftor\u2013ivacaftor (TEZ-IVA). In addition, the ELX-TEZ-IVA group showed significantly lower sweat chloride concentrations by 40.30\u2009mmol\/L (95% CI (\u221249.85, \u221230.74), p\u2009&lt;\u20090.00001). However, the incidence of adverse events in the ELX-TEZ-IVA group was slightly higher than that in the placebo, IVA, or TEZ-IVA groups.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>ELX-TEZ-IVA demonstrated efficacy in improving ppFEV1, CFQ-R RD, and sweat chloride concentrations in patients with CF. However, caution should be exercised regarding the incidence of AEs, particularly mild and moderate ones.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/20503121231225874","type":"journal-article","created":{"date-parts":[[2024,1,18]],"date-time":"2024-01-18T22:35:18Z","timestamp":1705617318000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":11,"title":["Elexacaftor\u2013tezacaftor\u2013ivacaftor for cystic fibrosis with Phe508del mutation: Evidence from randomized controlled trials"],"prefix":"10.1177","volume":"12","author":[{"given":"Rong","family":"He","sequence":"first","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Chengdu Medical College, Chengdu, China"},{"name":"Clinical Medical College, Chengdu Medical College, Chengdu, 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