{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T02:01:27Z","timestamp":1785290487790,"version":"3.55.0"},"reference-count":19,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2023,5,24]],"date-time":"2023-05-24T00:00:00Z","timestamp":1684886400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"publisher","award":["UL1TR002733"],"award-info":[{"award-number":["UL1TR002733"]}],"id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>In people with cystic fibrosis (pwCF), the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, such as Elexacaftor\u2013Tezacaftor\u2013Ivacaftor (ETI), on structural changes in the lungs is unclear.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To determine the impact of ETI on clinical parameters and on structural lung disease as measured by the changes in the chest computed tomography (CT) scans in pwCF.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Percent predicted forced expiratory volume in one second\u00a0(ppFEV1), body mass index (BMI), and microbiologic data were collected at initiation and 3\u2010month intervals for 1 year. Chest CT scans before starting ETI therapy (baseline) and at 1\u2010year on ETI therapy were compared by two pulmonologists independently.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The sample size was 67 pwCF, 30 (44.8%) males, median age of 25 (16, 33.5) years. Significant increases in ppFEV1 and BMI observed by 3 months of ETI therapy persisted throughout 1 year of ETI therapy (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001 at all\u2010time points for both). After 1 year on ETI, pwCF had significant reductions in <jats:italic>Pseudomona<\/jats:italic>s <jats:italic>aeruginosa<\/jats:italic> (\u221242%) and MRSA (\u221242%) positivity. None of the pwCF had worsening of chest CT parameters during 1 year of ETI therapy. Comparing chest CT findings at baseline and at 1\u2010year follow\u2010up, bronchiectasis was present in 65 (97%) pwCF and at 1\u2010year follow\u2010up decreased in 7 (11%). Bronchial wall thickening 64 (97%), decreased in 53 (79%). Mucous plugging in 63 (96%), absent in 11 (17%), and decreased in 50 (77%). Hyperinflation\/air trapping in 44 (67%), decreased in 11 (18%), absent in 27 (44%)<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>ETI significantly improved clinical outcomes and lung disease as documented by improvement in chest CT scans.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26485","type":"journal-article","created":{"date-parts":[[2023,5,24]],"date-time":"2023-05-24T11:43:09Z","timestamp":1684928589000},"page":"2308-2316","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Impact of Elexacaftor\u2013Tezacaftor\u2013Ivacaftor on lung disease in cystic fibrosis"],"prefix":"10.1002","volume":"58","author":[{"given":"Courtney","family":"Gushue","sequence":"first","affiliation":[{"name":"Departments of Pediatrics The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Division of Pulmonary Medicine The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariah","family":"Eisner","sequence":"additional","affiliation":[{"name":"Nationwide Children's Hospital Columbus Ohio USA"},{"name":"Biostatistics Resource, The Ohio State University College of Medicine Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shasha","family":"Bai","sequence":"additional","affiliation":[{"name":"Biostatistics Resource, The Ohio State University College of Medicine Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terri","family":"Johnson","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa","family":"Holtzlander","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Division of Pulmonary Medicine The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3483-0905","authenticated-orcid":false,"given":"Karen","family":"McCoy","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Division of Pulmonary Medicine The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0944-5076","authenticated-orcid":false,"given":"Shahid","family":"Sheikh","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Division of Pulmonary Medicine The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,5,24]]},"reference":[{"key":"e_1_2_12_2_1","unstructured":"US CF Foundation Johns Hopkins University the Hospital for Sick Children. 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