{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T07:50:23Z","timestamp":1780645823013,"version":"3.54.1"},"reference-count":22,"publisher":"MDPI AG","issue":"20","license":[{"start":{"date-parts":[[2022,10,19]],"date-time":"2022-10-19T00:00:00Z","timestamp":1666137600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["PROMISE-MCC18K0"],"award-info":[{"award-number":["PROMISE-MCC18K0"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["GORALS19Y5"],"award-info":[{"award-number":["GORALS19Y5"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["K23-HL138257-01A1"],"award-info":[{"award-number":["K23-HL138257-01A1"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["P30 DK065988"],"award-info":[{"award-number":["P30 DK065988"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Institute of Health","doi-asserted-by":"publisher","award":["PROMISE-MCC18K0"],"award-info":[{"award-number":["PROMISE-MCC18K0"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Institute of Health","doi-asserted-by":"publisher","award":["GORALS19Y5"],"award-info":[{"award-number":["GORALS19Y5"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Institute of Health","doi-asserted-by":"publisher","award":["K23-HL138257-01A1"],"award-info":[{"award-number":["K23-HL138257-01A1"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Institute of Health","doi-asserted-by":"publisher","award":["P30 DK065988"],"award-info":[{"award-number":["P30 DK065988"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>The availability of highly effective CFTR modulators is revolutionizing the treatment of cystic fibrosis (CF) and drastically improving outcomes. MRI-based imaging modalities are now emerging as highly sensitive endpoints, particularly in the setting of mild lung disease. Adult CF patients were recruited from a single center prior to starting treatment with E\/T\/I. The following studies were obtained before and after one month on treatment: spirometry, multiple breath nitrogen washout (MBW), 1H UTE MRI (structural images) and 19F MRI (ventilation images). Changes between visits were calculated, as were correlations between FEV1, lung clearance index (LCI), MRI structural scores, and MRI-based ventilation descriptors. Eight subjects had complete datasets for evaluation. Consistent with prior clinical trials, FEV1 and LCI improved after 28 days of E\/T\/I use. 1H UTE MRI detected improvements in bronchiectasis\/airway wall thickening score and mucus plugging score after 28 days of therapy. 19F MRI demonstrated improvements in fractional lung volume with slow gas washout time (FLV\u2191tau2) and ventilation defect percentage (VDP). Improvements in FLV\u2191tau2 and VDP correlated with improvement in FEV1 (r = 0.81 and 0.86, respectively, p &lt; 0.05). This observational study establishes the ability of 19F MRI and 1H UTE MRI to detect improvements in lung structure and function after E\/T\/I treatment. This study supports further development of 19F MRI and 1H UTE MRI as outcome measures for cystic fibrosis research and drug development.<\/jats:p>","DOI":"10.3390\/jcm11206160","type":"journal-article","created":{"date-parts":[[2022,10,19]],"date-time":"2022-10-19T22:19:53Z","timestamp":1666217993000},"page":"6160","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Dynamic Perfluorinated Gas MRI Shows Improved Lung Ventilation in People with Cystic Fibrosis after Elexacaftor\/Tezacaftor\/Ivacaftor: An Observational Study"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6785-5076","authenticated-orcid":false,"given":"Jennifer L.","family":"Goralski","sequence":"first","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"},{"name":"Marsico Lung Institute\/UNC Cystic Fibrosis Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"},{"name":"Division of Pediatric Pulmonology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0605-9420","authenticated-orcid":false,"given":"Sang Hun","family":"Chung","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering, University of North Carolina at Chapel Hill\u2014North Carolina State University, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agathe S.","family":"Ceppe","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"},{"name":"Marsico Lung Institute\/UNC Cystic Fibrosis Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3681-8851","authenticated-orcid":false,"given":"Margret Z.","family":"Powell","sequence":"additional","affiliation":[{"name":"Marsico Lung Institute\/UNC Cystic Fibrosis Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muthu","family":"Sakthivel","sequence":"additional","affiliation":[{"name":"Department of Radiology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian D.","family":"Handly","sequence":"additional","affiliation":[{"name":"Department of Radiology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1846-7680","authenticated-orcid":false,"given":"Yueh Z.","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Radiology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"},{"name":"Biomedical Research Imaging Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott H.","family":"Donaldson","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"},{"name":"Marsico Lung Institute\/UNC Cystic Fibrosis Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,10,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1016\/S0140-6736(19)32597-8","article-title":"Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: A double-blind, randomised, phase 3 trial","volume":"394","author":"Heijerman","year":"2019","journal-title":"Lancet"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1522","DOI":"10.1164\/rccm.202102-0509OC","article-title":"A Phase 3 Open-Label Study of Elexacaftor\/Tezacaftor\/Ivacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One F508del Allele","volume":"203","author":"Zemanick","year":"2021","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1097\/MCP.0000000000000732","article-title":"Monitoring early stage lung disease in cystic fibrosis","volume":"26","author":"Nissenbaum","year":"2020","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"S79","DOI":"10.1002\/ppul.24968","article-title":"Entering the era of highly effective modulator therapies","volume":"56","author":"Dave","year":"2021","journal-title":"Pediatr. Pulmonol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1016\/j.jcf.2020.08.015","article-title":"Outcomes and endpoints reported in studies of pulmonary exacerbations in people with cystic fibrosis: A systematic review","volume":"19","author":"McLeod","year":"2020","journal-title":"J. Cyst. Fibros."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1513\/pats.200611-183HT","article-title":"Advancing outcome measures for the new era of drug development in cystic fibrosis","volume":"4","author":"Ramsey","year":"2007","journal-title":"Proc. Am. Thorac. Soc."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"716","DOI":"10.1007\/s00330-006-0373-4","article-title":"Proton MRI appearance of cystic fibrosis: Comparison to CT","volume":"17","author":"Puderbach","year":"2007","journal-title":"Eur. Radiol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1513\/AnnalsATS.201501-021FR","article-title":"Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report","volume":"12","author":"Subbarao","year":"2015","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1463","DOI":"10.1164\/rccm.201710-1981ST","article-title":"Recommendations for a Standardized Pulmonary Function Report. An Official American Thoracic Society Technical Statement","volume":"196","author":"Culver","year":"2017","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1164\/ajrccm.159.1.9712108","article-title":"Spirometric reference values from a sample of the general U.S. population","volume":"159","author":"Hankinson","year":"1999","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"e133400","DOI":"10.1172\/jci.insight.133400","article-title":"Dynamic perfluorinated gas MRI reveals abnormal ventilation despite normal FEV1 in cystic fibrosis","volume":"5","author":"Goralski","year":"2020","journal-title":"JCI Insight"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1321","DOI":"10.1016\/j.ejrad.2011.02.045","article-title":"Morphologic and functional scoring of cystic fibrosis lung disease using MRI","volume":"81","author":"Eichinger","year":"2012","journal-title":"Eur. J. Radiol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1164\/rccm.202201-0219OC","article-title":"Effects of Elexacaftor\/Tezacaftor\/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles","volume":"206","author":"Graeber","year":"2022","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1923","DOI":"10.1513\/AnnalsATS.201603-203OC","article-title":"Ultrashort Echo-Time Magnetic Resonance Imaging Is a Sensitive Method for the Evaluation of Early Cystic Fibrosis Lung Disease","volume":"13","author":"Roach","year":"2016","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e122695","DOI":"10.1172\/jci.insight.122695","article-title":"Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR","volume":"3","author":"Donaldson","year":"2018","journal-title":"JCI Insight"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1028","DOI":"10.1002\/mrm.28457","article-title":"Comparison of single breath hyperpolarized (129) Xe MRI with dynamic (19) F MRI in cystic fibrosis lung disease","volume":"85","author":"McCallister","year":"2021","journal-title":"Magn. Reson. Med."},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Wucherpfennig, L., Triphan, S.M.F., Wege, S., Kauczor, H.U., Heussel, C.P., Schmitt, N., Wuennemann, F., Mayer, V.L., Sommerburg, O., and Mall, M.A. (2022). Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor\/tezacaftor\/ivacaftor therapy in adults with cystic fibrosis. J. Cyst. Fibros.","DOI":"10.1016\/j.jcf.2022.03.011"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1164\/rccm.201309-1659OC","article-title":"Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease","volume":"189","author":"Puderbach","year":"2014","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1016\/j.jcf.2018.05.003","article-title":"Multicentre standardisation of chest MRI as radiation-free outcome measure of lung disease in young children with cystic fibrosis","volume":"17","author":"Stahl","year":"2018","journal-title":"J. Cyst. Fibros."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1097\/RTI.0000000000000537","article-title":"Three-dimensional Ultrashort Echotime Magnetic Resonance Imaging for Combined Morphologic and Ventilation Imaging in Pediatric Patients With Pulmonary Disease","volume":"36","author":"Veldhoen","year":"2021","journal-title":"J. Thorac. Imaging"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"3811","DOI":"10.1007\/s00330-016-4218-5","article-title":"Lung morphology assessment of cystic fibrosis using MRI with ultra-short echo time at submillimeter spatial resolution","volume":"26","author":"Dournes","year":"2016","journal-title":"Eur. Radiol."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/20\/6160\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:57:11Z","timestamp":1760144231000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/20\/6160"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,10,19]]},"references-count":22,"journal-issue":{"issue":"20","published-online":{"date-parts":[[2022,10]]}},"alternative-id":["jcm11206160"],"URL":"https:\/\/doi.org\/10.3390\/jcm11206160","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,10,19]]}}}