{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T12:11:04Z","timestamp":1780315864598,"version":"3.54.1"},"reference-count":37,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T00:00:00Z","timestamp":1776643200000},"content-version":"vor","delay-in-days":19,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"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":[[2026,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Elexacaftor\/Tezacaftor\/Ivacaftor (ETI) therapy was approved in 2021 for clinical use in children with CF (cwCF) age 6\u201311 years, depending on their genetic variants. Our prior multi\u2010center study showed that lung T1\u2010MRI and multi\u2010breath washout (MBW) provided complementary pathobiological information on CF lung disease in cwCF 6\u201311 years of age. In this study, we hypothesized that lung T1\u2010MRI and MBW will sensitively detect lung improvements in cwCF after starting ETI therapy. To test this hypothesis, we obtained longitudinal lung T1\u2010MRI, MBW, and spirometry assessments in a cohort of cwCF 6\u201311 years of age before and after starting ETI therapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      CwCF (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200948, mean age\u2009=\u20098.6 years) were recruited from 3 cystic fibrosis (CF) centers (Riley Hospital for Children (Indianapolis, IN), University of Michigan, Michigan Medicine (Ann Arbor, MI), and Rainbow Babies and Children's Hospital (Cleveland, OH) for longitudinal lung T1\u2010MRI, MBW, and spirometry assessments before initiating ETI (baseline) as well as at 3\u2010months and 6\u2010months after initiating ETI. MBW and spirometry were performed according to published guidelines to obtain measurements of the lung clearance index (LCI) and percent predicted forced expiratory volume at 1\u2009s (ppFEV1), and percent predicted forced expiratory flow at 25%\u201375% (FEF\n                      <jats:sub>25<\/jats:sub>\n                      <jats:sub>%\u201375%<\/jats:sub>\n                      ), respectively. Lung T1\u2010MRI was used to obtain assessments of % normal lung perfusion (%NLP). An ANOVA analysis and descriptive statistics were used to compare the longitudinal changes in the lung T1\u2010MRI, MBW, and spirometry findings.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Across all subjects, significant improvements in both spirometry (ppFEV1, FEF\n                      <jats:sub>25<\/jats:sub>\n                      <jats:sub>%\u201375%<\/jats:sub>\n                      ) and MBW (LCI 2.5) were observed at 3\u2010months, and 6\u2010months post treatment (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). As a group, the lung T1\u2010MRI findings showed no significant changes despite a trend for improved lung perfusion. However, lung T1\u2010MRI did show a significant improvement in both 3\u2010months and 6\u2010months after starting ETI therapy (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.03) when evaluating only those subjects with mild\u2010moderate lung disease (ppFEV1 at baseline.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>MBW, spirometry, and lung T1\u2010MRI are all capable of detecting lung airway and perfusion changes in cwCF 6\u201311 years of age following the start of ETI therapy. MBW and spirometry assessments were able to detect a sustained improvement in pulmonary function testing regardless of baseline lung status, while lung T1\u2010MRI was able to detect significant improvements in lung perfusion in patients with more advanced lung disease. These findings, in combination with our prior cross\u2010sectional findings, suggest that MBW and lung T1\u2010MRI may provide complementary alternatives to chest CT and can be used to assess disease progression and\/or response to therapy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71628","type":"journal-article","created":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T08:51:27Z","timestamp":1776675087000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Lung T1\u2010MRI and Multi\u2010Breath Washout Detect Longitudinal Changes in Children 6\u201311 Years Old After Beginning Elexacaftor\/Tezacaftor\/Ivacaftor (ETI) Therapy"],"prefix":"10.1002","volume":"61","author":[{"given":"Samya Z.","family":"Nasr","sequence":"first","affiliation":[{"name":"Michigan Medicine University of Michigan Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1066-333X","authenticated-orcid":false,"given":"Chris A.","family":"Flask","sequence":"additional","affiliation":[{"name":"Case Western Reserve University Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2619-2894","authenticated-orcid":false,"given":"Madison E.","family":"Kretzler","sequence":"additional","affiliation":[{"name":"Case Western Reserve University Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James E.","family":"Slaven","sequence":"additional","affiliation":[{"name":"Indiana University School of Medicine Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aparna","family":"Joshi","sequence":"additional","affiliation":[{"name":"Michigan Medicine University of Michigan Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maryam Ghadimi","family":"Mahani","sequence":"additional","affiliation":[{"name":"Michigan Medicine University of Michigan Ann Arbor Michigan USA"},{"name":"Advanced Radiology Services Grand Rapids Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles","family":"Clem","sequence":"additional","affiliation":[{"name":"Riley Hospital for Children Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Cooper","sequence":"additional","affiliation":[{"name":"Riley Hospital for Children Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Farr","sequence":"additional","affiliation":[{"name":"Case Western Reserve University Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina J.","family":"MacAskill","sequence":"additional","affiliation":[{"name":"Case Western Reserve University Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amit","family":"Gupta","sequence":"additional","affiliation":[{"name":"Rainbow Babies and Children's Hospital University Hospitals \u2010 Cleveland Medical Center Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Ferrebee","sequence":"additional","affiliation":[{"name":"Rainbow Babies and Children's Hospital University Hospitals \u2010 Cleveland Medical Center Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9642-3814","authenticated-orcid":false,"given":"Kimberly A.","family":"McBennett","sequence":"additional","affiliation":[{"name":"Case Western Reserve University Cleveland Ohio USA"},{"name":"Rainbow Babies and Children's Hospital University Hospitals \u2010 Cleveland Medical Center Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4431-0644","authenticated-orcid":false,"given":"Clement L.","family":"Ren","sequence":"additional","affiliation":[{"name":"Perelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania USA"},{"name":"Children's Hospital of Philadelphia Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,4,20]]},"reference":[{"issue":"5","key":"e_1_2_11_2_1","doi-asserted-by":"crossref","first-page":"1229","DOI":"10.1164\/ajrccm.154.5.8912731","article-title":"Cystic Fibrosis","volume":"154","author":"Davis P. 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C. J.","year":"2020","journal-title":"Pediatric Pulmonology"},{"issue":"6","key":"e_1_2_11_20_1","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1001\/jamainternmed.2025.0505","article-title":"Projected Lifetime Cancer Risks From Current Computed Tomography Imaging","volume":"185","author":"Smith\u2010Bindman R.","year":"2025","journal-title":"JAMA Internal Medicine"},{"issue":"1","key":"e_1_2_11_21_1","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1186\/s41747-024-00502-9","article-title":"Low\u2010Dose High\u2010Resolution Chest Ct in Adults With Cystic Fibrosis: Intraindividual Comparison Between Photon\u2010Counting and Energy\u2010Integrating Detector CT","volume":"8","author":"Frings M.","year":"2024","journal-title":"European Radiology Experimental"},{"key":"e_1_2_11_22_1","doi-asserted-by":"crossref","unstructured":"R.Ward W. D.Carroll P.Cunningham et al. 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