{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T01:54:31Z","timestamp":1792806871312,"version":"4.5.0"},"reference-count":28,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2025,1,28]],"date-time":"2025-01-28T00:00:00Z","timestamp":1738022400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Deanship of Scientific Research at Northern Border University, Arar, KSA","award":["NBU-FFR-2025\u20131442-01"],"award-info":[{"award-number":["NBU-FFR-2025\u20131442-01"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background\/objectives: Cystic fibrosis (CF) is a life-limiting genetic disorder affecting multiple organ systems. This study compared clinical outcomes, hospitalization rates, and survival between children and adolescents with CF who received CFTR modulator therapies (ivacaftor, lumacaftor, tezacaftor, and elexacaftor). Methods: A retrospective cohort study was conducted using data from the TriNetX global collaborative network. Patients with CF aged 2\u201312 years (children) and 13\u201318 years (adolescents) who received CFTR modulator therapies were included. The propensity score matching balanced baseline characteristics between the two age groups. Results: After propensity score matching, 946 patients per group were analyzed. The incidence of respiratory failure (3.81% vs. 1.06%, p &lt; 0.001) and respiratory infections (62.7% vs. 57.5%, p = 0.021) were significantly higher in adolescents compared to children. Adolescents had a higher risk of respiratory failure (HR = 3.6, 95% CI = 1.79\u20137.21, p &lt; 0.001) and respiratory infections (HR = 1.09, 95% CI = 1.01\u20131.17, p &lt; 0.001). Adolescents also had a higher hospitalization rate (29.6% vs. 20.3%, p &lt; 0.001), with a 47% higher risk (HR = 1.47, 95% CI = 1.22\u20131.77, p = 0.001), more hospital visits per person (8.8 vs. 3.7, p = 0.004), and longer hospital stays (32.7 vs. 20.4 days, p = 0.006). Mortality rates were similar between the groups (1.58% vs. 1.26%, p = 0.56). Conclusions: CF patients who initiated CFTR modulator therapies during adolescence had a higher incidence of respiratory failure, respiratory infections, hospitalization rates, and healthcare resource utilization compared to those who started therapy in childhood, despite similar mortality rates. These findings highlight the importance of the early initiation of CFTR modulator therapies.<\/jats:p>","DOI":"10.3390\/children12020157","type":"journal-article","created":{"date-parts":[[2025,1,28]],"date-time":"2025-01-28T10:44:30Z","timestamp":1738061070000},"page":"157","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Clinical Outcomes in Patients with Cystic Fibrosis Receiving CFTR Modulators: A Comparison of Childhood Versus Adolescent Initiation"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9267-3787","authenticated-orcid":false,"given":"Eman A.","family":"Toraih","sequence":"first","affiliation":[{"name":"Department of Surgery, School of Medicine, Tulane University, New Orleans, LA 70112, USA"},{"name":"Department of Cardiovascular Perfusion, Interprofessional Research, College of Health Professions, SUNY Upstate Medical University, New York, NY 13210, USA"},{"name":"Medical Genetics Unit, Department of Histology and Cell Biology, Suez Canal University, Ismailia 41522, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-2031-8517","authenticated-orcid":false,"given":"Hassan A.","family":"Malik","sequence":"additional","affiliation":[{"name":"Louisiana State University Health Sciences Center, New Orleans School of Medicine, New Orleans, LA 70112, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rahib K.","family":"Islam","sequence":"additional","affiliation":[{"name":"Louisiana State University Health Sciences Center, New Orleans School of Medicine, New Orleans, LA 70112, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-6815-1896","authenticated-orcid":false,"given":"Humza A.","family":"Pirzadah","sequence":"additional","affiliation":[{"name":"Louisiana State University Health Sciences Center, New Orleans School of Medicine, New Orleans, LA 70112, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed","family":"Abdelmaksoud","sequence":"additional","affiliation":[{"name":"Department of Surgery, School of Medicine, Tulane University, New Orleans, LA 70112, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3381-2641","authenticated-orcid":false,"given":"Rami M.","family":"Elshazli","sequence":"additional","affiliation":[{"name":"Department of Surgery, School of Medicine, Tulane University, New Orleans, LA 70112, USA"},{"name":"Department of Biochemistry and Molecular Genetics, Faculty of Physical Therapy, Horus University, New Damietta 34517, Egypt"},{"name":"Department of Biological Sciences, Faculty of Science, New Mansoura University, New Mansoura City 35742, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul Antwi","family":"Boasiako","sequence":"additional","affiliation":[{"name":"College of Health Professions, SUNY Upstate Medical University, New York, NY 13210, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shehab Ahmed","family":"Alenazi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine, Northern Border University, Arar 91431, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelique","family":"Dabel","sequence":"additional","affiliation":[{"name":"College of Health Professions, SUNY Upstate Medical University, New York, NY 13210, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-3436-8889","authenticated-orcid":false,"given":"Jessan A.","family":"Jishu","sequence":"additional","affiliation":[{"name":"School of Medicine, Tulane University, New Orleans, LA 70112, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-2758-5581","authenticated-orcid":false,"given":"Bandar T.","family":"Alenezi","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Faculty of Medicine, Northern Border University, Arar 91431, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9191-7479","authenticated-orcid":false,"given":"Hani","family":"Aiash","sequence":"additional","affiliation":[{"name":"SUNY Upstate Medical University, New York, NY 13210, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1252-8403","authenticated-orcid":false,"given":"Manal S.","family":"Fawzy","sequence":"additional","affiliation":[{"name":"Center for Health Research, Northern Border University, Arar 91431, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,1,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Zampoli, M., Morrow, B.M., and Paul, G. 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Pharm."}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/12\/2\/157\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T10:37:53Z","timestamp":1759919873000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/12\/2\/157"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,28]]},"references-count":28,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2025,2]]}},"alternative-id":["children12020157"],"URL":"https:\/\/doi.org\/10.3390\/children12020157","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1,28]]}}}