{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T20:46:24Z","timestamp":1782161184826,"version":"3.54.5"},"reference-count":21,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2024,5,21]],"date-time":"2024-05-21T00:00:00Z","timestamp":1716249600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non\u2010eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the \u2018Vertex treatment\u2010Finder' on the Vertex\u00ae website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Among the study population (<jats:italic>N<\/jats:italic>\u2009=\u20091527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z\u2010score, CF\u2010associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, <jats:italic>p<\/jats:italic>\u2009=\u2009.010), lower median height z\u2010scores (\u22120.87 vs. \u22120.55, <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001), lower median body mass index z\u2010scores (\u22120.65 vs. \u22120.50, <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0\u20132] vs. 0 [IQR: 0\u20137], <jats:italic>p<\/jats:italic>\u2009=\u20090.001), and more non\u2010invasive ventilation support (2.6% vs. 0.9%, <jats:italic>p<\/jats:italic>\u2009=\u20090.008) than patients in group 1.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life\u2010changing drugs to improve clinical outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.27051","type":"journal-article","created":{"date-parts":[[2024,5,21]],"date-time":"2024-05-21T13:55:15Z","timestamp":1716299715000},"page":"2499-2506","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators"],"prefix":"10.1002","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6909-7993","authenticated-orcid":false,"given":"Halime","family":"Nay\u0131r B\u00fcy\u00fck\u015fahin","sequence":"first","affiliation":[{"name":"Division of Pulmonology, Department of Pediatrics Hacettepe University Faculty of Medicine Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1405-8401","authenticated-orcid":false,"given":"Nagehan","family":"Emiralio\u011flu","sequence":"additional","affiliation":[{"name":"Division of Pulmonology, Department of Pediatrics Hacettepe University Faculty of Medicine Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ebru","family":"Yal\u00e7\u0131n","sequence":"additional","affiliation":[{"name":"Division of Pulmonology, Department of Pediatrics Hacettepe University Faculty of Medicine Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Velat","family":"\u015een","sequence":"additional","affiliation":[{"name":"Division of Pulmonology, Department of Pediatrics Dicle University Faculty of Medicine Diyarbak\u0131r Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hadice","family":"Selimo\u011flu \u015een","sequence":"additional","affiliation":[{"name":"Department of Pulmonology Dicle University Faculty of Medicine Diyarbak\u0131r Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00fcseyin","family":"Arslan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Pulmonology, Department of Pediatrics, Cerrahpa\u015fa Medicine Faculty Istanbul University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Azer K\u0131l\u0131\u00e7","family":"Ba\u015fkan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Pulmonology, Department of Pediatrics, Cerrahpa\u015fa Medicine Faculty Istanbul University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatma Bet\u00fcl","family":"\u00c7ak\u0131r","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Faculty of Medicine Bezmialem University Istanbul 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