{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,12]],"date-time":"2026-07-12T05:07:37Z","timestamp":1783832857914,"version":"3.55.0"},"reference-count":43,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T00:00:00Z","timestamp":1777852800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T00:00:00Z","timestamp":1777852800000},"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"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Magnetic Resonance Imaging"],"published-print":{"date-parts":[[2026,8]]},"abstract":"ABSTRACT<\/jats:title>\n \n Background<\/jats:title>\n \n Ultrashort echo time (UTE) MRI overcomes the low signal intensity and short T\n 2<\/jats:sub>\n * of pulmonary tissues, improving image quality. Abnormally low UTE signal intensities are associated with hallmarks of cystic fibrosis (CF), including gas trapping and lung hyperexpansion.\n <\/jats:p>\n <\/jats:sec>\n \n Purpose<\/jats:title>\n To explore short\u2010term repeatability and sensitivity to treatment of low signal volume (LSV) from UTE MRI in pediatric CF.<\/jats:p>\n <\/jats:sec>\n \n Study\u2010Type<\/jats:title>\n Single\u2010site, retrospective, longitudinal.<\/jats:p>\n <\/jats:sec>\n \n Subjects<\/jats:title>\n Thirteen participants with stable CF (6M\/7F, median age\u2009=\u200915\u2009years old) were scanned at baseline and 1\u2010month to evaluate short\u2010term repeatability. Subsequently, 14 CF participants (7M\/7F median age\u2009=\u200916\u2009years old) were scanned pre\u2010 and 1\u2010month post\u2010initiation of elexacaftor\/tezacaftor\/ivacaftor (ETI).<\/jats:p>\n <\/jats:sec>\n \n Field Strength\/Sequence<\/jats:title>\n Three\u2010dimensional stack\u2010of\u2010spirals for UTE, 2\u2010dimensional gradient\u2010echo for hyperpolarized xenon (Xe\u2010MRI), 3\u2010dimensional gradient\u2010echo for thoracic cavity estimation at 3\u2009T.<\/jats:p>\n <\/jats:sec>\n \n Assessment<\/jats:title>\n LSV was analyzed from UTE MRI. Same\u2010day spirometry, multiple\u2010breath washout, and Xe\u2010MRI were also performed to compare to LSV.<\/jats:p>\n <\/jats:sec>\n \n Statistical Tests<\/jats:title>\n \n Differences were assessed with the Wilcoxon matched\u2010pairs signed\u2010rank test. Bland\u2013Altman analysis and the Intraclass Correlation Coefficient (ICC) were used to assess 1\u2010month repeatability in stable CF. Relationships between measures were assessed with Spearman correlation.\n p<\/jats:italic>\n \u2009<\u20090.05 was considered significant.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Baseline LSV was correlated with forced expiratory volume in 1 s (FEV\n 1<\/jats:sub>\n ), FEV\n 1<\/jats:sub>\n to forced vital capacity ratio (FEV\n 1<\/jats:sub>\n \/FVC), lung clearance index (LCI), and ventilation defect percent (VDP) (all |\n \u03c1<\/jats:italic>\n |\u2009\u2265\u20090.50). LSV was not significantly different after 1\u2010month (20.1 [10.9\u201325.5]% vs. 21.7 [12.7\u201328.2]%,\n p<\/jats:italic>\n \u2009=\u20090.4548) in stable pediatric CF with Bland\u2013Altman bias <\u20091% and ICC\u2009=\u20090.93. LSV was significantly reduced from 18.8 [12.0\u201330.3]% to 16.4 [7.8\u201319.6]% after 1\u2010month of ETI and correlated with absolute differences in FEV\n 1<\/jats:sub>\n , FEV\n 1<\/jats:sub>\n \/FVC, LCI, and VDP (all |\n \u03c1<\/jats:italic>\n |\u2009\u2265\u20090.59).\n <\/jats:p>\n <\/jats:sec>\n \n Data Conclusion<\/jats:title>\n LSV analysis was feasible and repeatable in pediatric CF over a 1\u2010month period. LSV was significantly reduced 1\u2010month after ETI treatment, indicating sensitivity to reduced gas trapping, hyperexpansion, and obstruction.<\/jats:p>\n <\/jats:sec>\n \n Evidence Level<\/jats:title>\n 4.<\/jats:p>\n <\/jats:sec>\n \n Technical Efficacy<\/jats:title>\n 1.<\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/jmri.70333","type":"journal-article","created":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T12:44:08Z","timestamp":1777898648000},"page":"442-451","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Automated Ultrashort Echo Time (\n UTE<\/scp>\n )\n MRI<\/scp>\n Low Signal Volume Analysis in Stable Pediatric Cystic Fibrosis and After Elexacaftor\/Tezacaftor\/Ivacaftor Therapy"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5645-0180","authenticated-orcid":false,"given":"Brandon","family":"Zanette","sequence":"first","affiliation":[{"name":"Translational Medicine 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