{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T11:49:50Z","timestamp":1782474590250,"version":"3.54.5"},"reference-count":62,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T00:00:00Z","timestamp":1779926400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T00:00:00Z","timestamp":1779926400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Cystic fibrosis transmembrane conductance regulator modulators (CFTRm) represent a breakthrough in the treatment of people with cystic fibrosis (PwCF), offering improvements in health outcomes and quality of life (QoL) previously unattainable.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To descriptively evaluate 12\u2010month outcomes during treatment with three CFTRm therapies in a real\u2010world cohort of children and adults with CF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This prospective, observational study included 146 PwCF aged 2\u201356\u2009years treated at two Polish CF centres. Patients received lumacaftor\/ivacaftor (LUM\/IVA), tezacaftor\/ivacaftor (TEZ\/IVA) or elexacaftor\/tezacaftor\/ivacaftor (ELX\/TEZ\/IVA). Assessments were conducted at baseline and every 3 months over 12\u2009months.<\/jats:p>\n                    <jats:p>Parameters evaluated included spirometry, impulse oscillometry (IOS), lung clearance index (LCI), anthropometric data, serum vitamin D3, sweat chloride (SwCl), lung imaging (Brasfield score), general clinical status \u2013 Schwachman\u2010Kulczycki (S\u2010K score), and quality of life (QoL). Statistical analyses were performed using the software R (v. 4.4.1).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Most parameters improved over time, with response magnitude varying by treatment. Significant improvements were observed in forced expiratory volume in 1\u2009s (FEV\n                      <jats:sub>1<\/jats:sub>\n                      ), forced vital capacity (FVC), maximal expiratory flow at 50% of vital capacity (MEF\n                      <jats:sub>50<\/jats:sub>\n                      ), LCI, most IOS parameters, SwCl, anthropometric parameters, Brasfield and S\u2010K scores. Vitamin D3 concentrations and resistance at 5\u2009Hz (R5) did not change significantly. All therapies led to better QoL.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>CFTRm \u2013 particularly ELX\/TEZ\/IVA \u2013 demonstrated substantial clinical benefits across age groups. The treatment effect varied over time in the majority of patients. Different CFTRm's impact on respiratory function, nutritional status, and well\u2010being underscores the importance of personalized approaches in CF care.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71681","type":"journal-article","created":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T13:11:59Z","timestamp":1779973919000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Comparison of Long Term Effects of Treatment of Different CFTR Modulators in People With Cystic Fibrosis"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-7547-3383","authenticated-orcid":false,"given":"Paulina","family":"Famulska","sequence":"first","affiliation":[{"name":"Department of Paediatrics and Cystic Fibrosis Polanki Children's Hospital Gda\u0144sk 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