{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T04:35:04Z","timestamp":1791952504992,"version":"4.3.4"},"reference-count":41,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,12,9]],"date-time":"2022-12-09T00:00:00Z","timestamp":1670544000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000897","name":"CF Foundation","doi-asserted-by":"publisher","award":["MCCOY19RO\u2013Core C [Kopp]"],"award-info":[{"award-number":["MCCOY19RO\u2013Core C [Kopp]"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 HL155095"],"award-info":[{"award-number":["R01 HL155095"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 HL148171"],"award-info":[{"award-number":["R01 HL148171"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 HL158747"],"award-info":[{"award-number":["R01 HL158747"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2023,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Cystic fibrosis (CF) is a multisystem disease with progressive deterioration. Recently, CF transmembrane conductance regulator (CFTR) modulator therapies were introduced that repair underlying protein defects. Objective of this study was to determine the impact of elexacaftor\u2013tezacaftor\u2013ivacaftor (ETI) on clinical parameters and inflammatory responses in people with CF (pwCF).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Lung function (FEV<jats:sub>1<\/jats:sub>), body mass index (BMI) and microbiologic data were collected at initiation and 3\u2010month intervals for 1 year. Blood was analyzed at baseline and 6 months for cytokines and immune cell populations via flow cytometry and compared to non\u2010CF controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sample size was 48 pwCF, 28 (58.3%) males with a mean age of 28.8\u2009\u00b1\u200910.7 years. Significant increases in %predicted FEV<jats:sub>1<\/jats:sub> and BMI were observed through 6 months of ETI therapy with no change thereafter. Changes in FEV<jats:sub>1<\/jats:sub> and BMI at 3 months were significantly correlated (<jats:italic>r<\/jats:italic>\u2009=\u200957.2, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01). There were significant reductions in <jats:italic>Pseudomona<\/jats:italic>s and <jats:italic>Staphylococcus<\/jats:italic> positivity (percent of total samples) in pwCF through 12 months of ETI treatment. Healthy controls (<jats:italic>n<\/jats:italic>\u2009=\u200920) had significantly lower levels of circulating neutrophils,\u00a0interleukin (IL)\u20106, IL\u20108, and IL\u201017A and higher levels of IL\u201013 compared to pwCF at baseline (<jats:italic>n<\/jats:italic>\u2009=\u200948). After 6 months of ETI, pwCF had significant decreases in IL\u20108, IL\u20106, and IL\u201017A levels and normalization of peripheral blood immune cell composition.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In pwCF, ETI significantly improved clinical outcomes, reduced systemic pro\u2010inflammatory cytokines, and restored circulating immune cell composition after 6 months of therapy.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26261","type":"journal-article","created":{"date-parts":[[2022,11,29]],"date-time":"2022-11-29T10:04:11Z","timestamp":1669716251000},"page":"825-833","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":93,"title":["Impact of elexacaftor\u2013tezacaftor\u2013ivacaftor on bacterial colonization and inflammatory responses in cystic fibrosis"],"prefix":"10.1002","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0944-5076","authenticated-orcid":false,"given":"Shahid","family":"Sheikh","sequence":"first","affiliation":[{"name":"Department of Pediatrics The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Division of Pulmonary Medicine Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"Rodney D.","family":"Britt","sequence":"additional","affiliation":[{"name":"Department of Pediatrics The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Center for Perinatal Research The Abigail Wexner Research Institute at Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nancy A.","family":"Ryan\u2010Wenger","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aiman Q.","family":"Khan","sequence":"additional","affiliation":[{"name":"Center for Perinatal Research The Abigail Wexner Research Institute at Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brandon W.","family":"Lewis","sequence":"additional","affiliation":[{"name":"Center for Perinatal Research The Abigail Wexner Research Institute at Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Courtney","family":"Gushue","sequence":"additional","affiliation":[{"name":"Department of Pediatrics The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Division of Pulmonary Medicine Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hazel","family":"Ozuna","sequence":"additional","affiliation":[{"name":"Center for Microbial Pathogenesis The Abigail Wexner Research Institute at Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Devi","family":"Jaganathan","sequence":"additional","affiliation":[{"name":"Center for Microbial Pathogenesis The Abigail Wexner Research Institute at 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":"Department of Pediatrics The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Division of Pulmonary Medicine Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin T.","family":"Kopp","sequence":"additional","affiliation":[{"name":"Department of Pediatrics The Ohio State University College of Medicine Columbus Ohio USA"},{"name":"Division of Pulmonary Medicine Nationwide Children's Hospital Columbus Ohio USA"},{"name":"Center for Microbial Pathogenesis The Abigail Wexner Research Institute at Nationwide Children's Hospital Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,12,9]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24129"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1385\/CRIAI:23:1:005"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-007-8039-9"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1186\/1465-9921-4-8"},{"key":"e_1_2_10_6_1","first-page":"57","volume-title":"Cystic Fibrosis","author":"Chmiel 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