{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,6]],"date-time":"2026-06-06T11:02:17Z","timestamp":1780743737910,"version":"3.54.1"},"reference-count":30,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T00:00:00Z","timestamp":1769644800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T00:00:00Z","timestamp":1769644800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["MOP 123431"],"award-info":[{"award-number":["MOP 123431"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["PJT 153099"],"award-info":[{"award-number":["PJT 153099"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006126","name":"Hospital for Sick Children","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006126","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Magnetic Resonance Imaging"],"published-print":{"date-parts":[[2026,6]]},"abstract":"ABSTRACT<\/jats:title>\n \n Background<\/jats:title>\n \n Elexacaftor\/tezacaftor\/ivacaftor (ETI) is a current standard therapy for pediatric cystic fibrosis (CF). Multiple\u2010breath washout\n 129<\/jats:sup>\n Xe MRI (MBW Xe\u2010MRI) is improved following 1\u2009month of treatment. However, the utility of MBW Xe\u2010MRI over extended ETI treatment and its comparison to single\u2010breath Xe\u2010MRI and pulmonary function tests (PFTs) in monitoring disease progression remains unclear.\n <\/jats:p>\n <\/jats:sec>\n \n Purpose<\/jats:title>\n To compare MBW Xe\u2010MRI and single\u2010breath Xe\u2010MRI in a small pediatric CF cohort at 1, 6, 12, and 24\u2009months post\u2010ETI initiation.<\/jats:p>\n <\/jats:sec>\n \n Study Type<\/jats:title>\n Prospective longitudinal cohort study.<\/jats:p>\n <\/jats:sec>\n \n Subjects<\/jats:title>\n 14 participants (7 female, median age 15.5 [14, 17] years) with CF undergoing ETI.<\/jats:p>\n <\/jats:sec>\n \n Field Strength\/Sequence<\/jats:title>\n Xe\u2010MRI using a gradient echo sequence at 3T.<\/jats:p>\n <\/jats:sec>\n \n Assessment<\/jats:title>\n \n A total of 12 participants completed MBW Xe\u2010MRI, single\u2010breath Xe\u2010MRI, and PFTs (spirometry, N\n 2<\/jats:sub>\n MBW) at \u2265\u20092 of 4 visits (1, 6, 12, and 24\u2009months post\u2010ETI). Fractional ventilation (FV) and FV coefficient of variation (CoV\n FV<\/jats:sub>\n ) maps were calculated from MBW Xe\u2010MRI. Ventilation defect percent (VDP) was calculated from single\u2010breath Xe\u2010MRI.\n <\/jats:p>\n <\/jats:sec>\n \n Statistical Tests<\/jats:title>\n \n Longitudinal changes were analyzed using a linear mixed\u2010effects model (fixed effect: time, random intercept: participant). Significance via ANOVA F\u2010test,\n p<\/jats:italic>\n \u2009<\u20090.05. Intra\u2010class correlation coefficients (ICC) were used to quantify between\u2010 and within\u2010subject variability.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Data completeness (total number of acquired data points divided by expected data points across 14 participants, 4 visits) was \u2265\u200975%. While PFTs\/VDP remained stable over 24\u2009months (ICC \u2265\u20090.93; linear mixed\u2010effects model of time effect for ppFEV\n 1<\/jats:sub>\n , LCI and VDP was not significant with\n p<\/jats:italic>\n \u2009=\u20090.68, 0.13 and 0.12, respectively), CoV\n FV<\/jats:sub>\n demonstrated a small but significant increase (slope magnitude +0.001\/month). Furthermore, two participants had elevated CoV\n FV<\/jats:sub>\n despite normal VDP. Finally, MBW Xe\u2010MRI metrics showed higher within\u2010subject variability than PFTs\/VDP (ICC: FV\u2009=\u20090.41, CoV\n FV<\/jats:sub>\n \u2009=\u20090.55 vs. VDP\/PFTs \u2265\u20090.92).\n <\/jats:p>\n <\/jats:sec>\n \n Data Conclusion<\/jats:title>\n \n CoV\n FV<\/jats:sub>\n may continue to evolve over 2\u2009years in pediatric CF patients receiving ETI, particularly in individuals with persistent ventilation defects.\n <\/jats:p>\n <\/jats:sec>\n \n Evidence Level<\/jats:title>\n 1.<\/jats:p>\n <\/jats:sec>\n \n Stage of Technical Efficacy<\/jats:title>\n 2.<\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/jmri.70242","type":"journal-article","created":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T12:41:16Z","timestamp":1769690476000},"page":"1652-1661","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Comparison of Single\u2010Breath and Multi\u2010Breath Xe\u2010\n MRI<\/scp>\n in the Longitudinal Assessment of Treatment in Children With Cystic Fibrosis"],"prefix":"10.1002","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4756-7536","authenticated-orcid":false,"given":"Faiyza S.","family":"Alam","sequence":"first","affiliation":[{"name":"Department of Medical Biophysics University of Toronto Toronto Ontario Canada"},{"name":"Translational Medicine Program The Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-7053-0504","authenticated-orcid":false,"given":"Samal","family":"Munidasa","sequence":"additional","affiliation":[{"name":"Department of Medical Biophysics University of Toronto Toronto Ontario Canada"},{"name":"Translational Medicine Program The Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brandon","family":"Zanette","sequence":"additional","affiliation":[{"name":"Translational Medicine Program The Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Li","sequence":"additional","affiliation":[{"name":"Translational Medicine Program The Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon","family":"Braganza","sequence":"additional","affiliation":[{"name":"Translational Medicine Program The Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4057-6592","authenticated-orcid":false,"given":"Felix","family":"Ratjen","sequence":"additional","affiliation":[{"name":"Translational Medicine Program The Hospital for Sick Children Toronto Ontario Canada"},{"name":"Division of Respirology The Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7396-188X","authenticated-orcid":false,"given":"Giles","family":"Santyr","sequence":"additional","affiliation":[{"name":"Department of Medical Biophysics University of Toronto Toronto Ontario Canada"},{"name":"Translational Medicine Program The Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,1,29]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.12.009"},{"key":"e_1_2_9_3_1","volume-title":"2023 Patient Registry Annual Data Report","year":"2024"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2016.07.008"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.29566"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1513\/annalsats.202501\u2010028oc"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/jmri.29605"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1569-1993(23)01062-7"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.10206"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.27234"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.28099"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/jmri.28638"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.09.021"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201908\u20101590ST"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00069712"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl\u20102021\u2010218347"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202301\u20100021oc"},{"key":"e_1_2_9_18_1","unstructured":"J.Scheller M.Gutberlet andA. 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