{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T15:34:35Z","timestamp":1791646475959,"version":"4.3.4"},"reference-count":51,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2022,8,1]],"date-time":"2022-08-01T00:00:00Z","timestamp":1659312000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"independent medical grant from Vertex Pharmaceuticals Incorporated","award":["IIS-2018-107555"],"award-info":[{"award-number":["IIS-2018-107555"]}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["82DZL009B1"],"award-info":[{"award-number":["82DZL009B1"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["82DZL002A1"],"award-info":[{"award-number":["82DZL002A1"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["82DZL005B1"],"award-info":[{"award-number":["82DZL005B1"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["82DZL004B1"],"award-info":[{"award-number":["82DZL004B1"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["CRC 1449 \u2013 431232613 Z02"],"award-info":[{"award-number":["CRC 1449 \u2013 431232613 Z02"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"independent medical grant from Vertex Pharmaceuticals Incorporated","award":["IIS-2018-107555"],"award-info":[{"award-number":["IIS-2018-107555"]}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["82DZL009B1"],"award-info":[{"award-number":["82DZL009B1"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["82DZL002A1"],"award-info":[{"award-number":["82DZL002A1"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["82DZL005B1"],"award-info":[{"award-number":["82DZL005B1"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["82DZL004B1"],"award-info":[{"award-number":["82DZL004B1"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["CRC 1449 \u2013 431232613 Z02"],"award-info":[{"award-number":["CRC 1449 \u2013 431232613 Z02"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.atsjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,8,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>We recently demonstrated that triple-combination CFTR (cystic fibrosis transmembrane conductance regulator) modulator therapy with elexacaftor\/tezacaftor\/ivacaftor (ELX\/TEZ\/IVA) improves CFTR function in airway and intestinal epithelia to 40\u201350% of normal in patients with cystic fibrosis (CF) with one or two F508del alleles. In previous studies, this improvement of CFTR function was shown to improve clinical outcomes; however, effects on the lung clearance index (LCI) determined by multiple-breath washout and abnormalities in lung morphology and perfusion detected by magnetic resonance imaging (MRI) have not been studied.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To examine the effect of ELX\/TEZ\/IVA on LCI and lung MRI scores in patients with CF and one or two F508del alleles aged \u2a7e12 years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This prospective, observational, multicenter, postapproval study assessed LCI and lung MRI scores before and 8\u201316 weeks after initiation of ELX\/TEZ\/IVA.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Measurements and Main Results<\/jats:title>\n                    <jats:p>A total of 91 patients with CF, including 45 heterozygous for F508del and a minimal function mutation (MF) and 46 homozygous for F508del, were enrolled in this study. Treatment with ELX\/TEZ\/IVA improved LCI in F508del\/MF (\u22122.4; interquartile range [IQR], \u22123.7 to \u22121.1; P\u2009&amp;lt;\u20090.001) and F508del homozygous (\u22121.4; IQR, \u22122.4 to \u22120.4; P\u2009&amp;lt;\u20090.001) patients. Furthermore, ELX\/TEZ\/IVA improved the MRI global score in F508del\/MF (\u22126.0; IQR, \u221211.0 to \u22121.3; P\u2009&amp;lt;\u20090.001) and F508del homozygous (\u22126.5; IQR, \u221211.0 to \u22121.3; P\u2009&amp;lt;\u20090.001) patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Our data demonstrate that improvement of CFTR function by ELX\/TEZ\/IVA improves lung ventilation and abnormalities in lung morphology, including airway mucus plugging and wall thickening, in adolescent and adult patients with CF and one or two F508del alleles in a real-world, postapproval setting.<\/jats:p>\n                    <jats:p>Clinical trial registered with www.clinicaltrials.gov (NCT04732910).<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1164\/rccm.202201-0219oc","type":"journal-article","created":{"date-parts":[[2022,5,10]],"date-time":"2022-05-10T11:04:25Z","timestamp":1652180665000},"page":"311-320","update-policy":"https:\/\/doi.org\/10.1164\/crossmarkpolicies","source":"Crossref","is-referenced-by-count":118,"title":["Effects of Elexacaftor\/Tezacaftor\/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two\n                    <i>F508del<\/i>\n                    Alleles"],"prefix":"10.1093","volume":"206","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1666-8924","authenticated-orcid":false,"given":"Simon Y.","family":"Graeber","sequence":"first","affiliation":[{"name":"Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and"},{"name":"Berlin Institute of Health at Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, Berlin, Germany"},{"name":"German Center for Lung Research (DZL), associated partner site, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diane M.","family":"Renz","sequence":"additional","affiliation":[{"name":"Department for Radiology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6941-8315","authenticated-orcid":false,"given":"Mirjam","family":"Stahl","sequence":"additional","affiliation":[{"name":"Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and"},{"name":"Berlin Institute of Health at Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, Berlin, Germany"},{"name":"German Center for Lung Research (DZL), associated partner site, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophia T.","family":"Pallenberg","sequence":"additional","affiliation":[{"name":"Department for Pediatric Pneumology, Allergology, and Neonatology"},{"name":"German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease (BREATH)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olaf","family":"Sommerburg","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics"},{"name":"Department of Translational Pulmonology, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lutz","family":"Naehrlich","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany; and"},{"name":"Universities of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL), Giessen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian","family":"Berges","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics"},{"name":"Department of Translational Pulmonology, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martha","family":"Dohna","sequence":"additional","affiliation":[{"name":"Department for Radiology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix C.","family":"Ringshausen","sequence":"additional","affiliation":[{"name":"German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease (BREATH)"},{"name":"Department for Pneumology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"Doellinger","sequence":"additional","affiliation":[{"name":"Department of Radiology, Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt-Universit\u00e4t zu Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constanze","family":"Vitzthum","sequence":"additional","affiliation":[{"name":"Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and"},{"name":"German Center for Lung Research (DZL), associated partner site, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jobst","family":"R\u00f6hmel","sequence":"additional","affiliation":[{"name":"Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and"},{"name":"German Center for Lung Research (DZL), associated partner site, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Allomba","sequence":"additional","affiliation":[{"name":"Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and"},{"name":"German Center for Lung Research (DZL), associated partner site, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne","family":"H\u00e4mmerling","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Barth","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany; and"},{"name":"Universities of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL), Giessen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"R\u00fcckes-Nilges","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany; and"},{"name":"Universities of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL), Giessen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6962-037X","authenticated-orcid":false,"given":"Mark O.","family":"Wielp\u00fctz","sequence":"additional","affiliation":[{"name":"Department of Translational Pulmonology, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), and"},{"name":"Department of Diagnostic and Interventional Radiology, University of Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gesine","family":"Hansen","sequence":"additional","affiliation":[{"name":"Department for Pediatric Pneumology, Allergology, and Neonatology"},{"name":"German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease (BREATH)"},{"name":"Cluster of Excellence RESIST (EXC 2155), German Research Foundation (DFG), Hannover Medical School, Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Vogel-Claussen","sequence":"additional","affiliation":[{"name":"Department for Radiology"},{"name":"Department for Pediatric Pneumology, Allergology, and Neonatology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Burkhard","family":"T\u00fcmmler","sequence":"additional","affiliation":[{"name":"Department for Pediatric Pneumology, Allergology, and Neonatology"},{"name":"German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease (BREATH)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4057-2199","authenticated-orcid":false,"given":"Marcus A.","family":"Mall","sequence":"additional","affiliation":[{"name":"Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and"},{"name":"Berlin Institute of Health at Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, Berlin, Germany"},{"name":"German Center for Lung Research (DZL), associated partner site, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9582-1025","authenticated-orcid":false,"given":"Anna-Maria","family":"Dittrich","sequence":"additional","affiliation":[{"name":"Department for Pediatric Pneumology, Allergology, and Neonatology"},{"name":"German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease (BREATH)"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,8,1]]},"reference":[{"key":"2026022021141971100_bib1","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":"2026022021141971100_bib2","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":"2026022021141971100_bib3","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1164\/rccm.202008-3176LE","article-title":"Safety and efficacy of elexacaftor\/tezacaftor\/ivacaftor for 24 weeks or longer in people with cystic fibrosis and one or more F508del alleles: interim results of an open-label phase 3 clinical trial","volume":"203","author":"Griese","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib4","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1056\/NEJMoa2100665","article-title":"Triple therapy for cystic fibrosis Phe508del-gating and -residual function genotypes","volume":"385","author":"Barry","year":"2021","journal-title":"N Engl J Med"},{"key":"2026022021141971100_bib5","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1016\/S2213-2600(21)00454-9","article-title":"Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial","volume":"10","author":"Sutharsan","year":"2022","journal-title":"Lancet Respir Med"},{"key":"2026022021141971100_bib6","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1164\/rccm.202108-1986OC","article-title":"Clinical effectiveness of elexacaftor\/tezacaftor\/ivacaftor in people with cystic fibrosis: a clinical trial","volume":"205","author":"Nichols","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib7","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1164\/rccm.202011-4153OC","article-title":"Rapid improvement after starting elexacaftor-tezacaftor-ivacaftor in patients with cystic fibrosis and advanced pulmonary disease","volume":"204","author":"Burgel","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib8","doi-asserted-by":"crossref","first-page":"2519","DOI":"10.1016\/S0140-6736(16)00576-6","article-title":"Cystic fibrosis","volume":"388","author":"Elborn","year":"2016","journal-title":"Lancet"},{"key":"2026022021141971100_bib9","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/S2213-2600(19)30337-6","article-title":"The future of cystic fibrosis care: a global perspective","volume":"8","author":"Bell","year":"2020","journal-title":"Lancet Respir Med"},{"key":"2026022021141971100_bib10","doi-asserted-by":"crossref","first-page":"1193","DOI":"10.1164\/rccm.201910-1943SO","article-title":"Cystic fibrosis: emergence of highly effective targeted therapeutics and potential clinical implications","volume":"201","author":"Mall","year":"2020","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib11","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1164\/rccm.202110-2249OC","article-title":"Effects of elexacaftor\/tezacaftor\/ivacaftor therapy on CFTR function in patients with cystic fibrosis and one or two F508del alleles","volume":"205","author":"Graeber","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib12","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1056\/NEJMoa1409547","article-title":"Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR","volume":"373","author":"Wainwright","year":"2015","journal-title":"N Engl J Med"},{"key":"2026022021141971100_bib13","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/S2213-2600(16)30427-1","article-title":"Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study","volume":"5","author":"Konstan","year":"2017","journal-title":"Lancet Respir Med"},{"key":"2026022021141971100_bib14","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1164\/rccm.201710-1983OC","article-title":"Effects of lumacaftor-ivacaftor therapy on cystic fibrosis transmembrane conductance regulator function in Phe508del homozygous patients with cystic fibrosis","volume":"197","author":"Graeber","year":"2018","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib15","doi-asserted-by":"crossref","first-page":"2013","DOI":"10.1056\/NEJMoa1709846","article-title":"Tezacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del","volume":"377","author":"Taylor-Cousar","year":"2017","journal-title":"N Engl J Med"},{"key":"2026022021141971100_bib16","doi-asserted-by":"crossref","first-page":"2024","DOI":"10.1056\/NEJMoa1709847","article-title":"Tezacaftor-ivacaftor in residual-function heterozygotes with cystic fibrosis","volume":"377","author":"Rowe","year":"2017","journal-title":"N Engl J Med"},{"key":"2026022021141971100_bib17","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1164\/rccm.201704-0717OC","article-title":"Tezacaftor\/ivacaftor in subjects with cystic fibrosis and F508del\/F508del-CFTR or F508del\/G551D-CFTR","volume":"197","author":"Donaldson","year":"2018","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib18","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1016\/S2213-2600(20)30510-5","article-title":"Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study","volume":"9","author":"Flume","year":"2021","journal-title":"Lancet Respir Med"},{"key":"2026022021141971100_bib19","doi-asserted-by":"crossref","first-page":"912","DOI":"10.1164\/rccm.201608-1754OC","article-title":"Lumacaftor\/ivacaftor in patients aged 6-11 years with cystic fibrosis and homozygous for F508del-CFTR","volume":"195","author":"Milla","year":"2017","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib20","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1016\/S2213-2600(17)30215-1","article-title":"Efficacy and safety of lumacaftor and ivacaftor in patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR: a randomised, placebo-controlled phase 3 trial","volume":"5","author":"Ratjen","year":"2017","journal-title":"Lancet Respir Med"},{"key":"2026022021141971100_bib21","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1513\/AnnalsATS.202008-1054OC","article-title":"Effects of lumacaftor-ivacaftor on lung clearance index, magnetic resonance imaging, and airway microbiome in Phe508del homozygous patients with cystic fibrosis","volume":"18","author":"Graeber","year":"2021","journal-title":"Ann Am Thorac Soc"},{"key":"2026022021141971100_bib22","doi-asserted-by":"crossref","first-page":"931","DOI":"10.1016\/j.jcf.2020.05.010","article-title":"Changes in LCI in F508del\/F508del patients treated with lumacaftor\/ivacaftor: results from the prospect study","volume":"19","author":"Shaw","year":"2020","journal-title":"J Cyst Fibros"},{"key":"2026022021141971100_bib23","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1136\/thx.2009.125831","article-title":"Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function","volume":"65","author":"Amin","year":"2010","journal-title":"Thorax"},{"key":"2026022021141971100_bib24","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1183\/09031936.00072510","article-title":"The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis","volume":"37","author":"Amin","year":"2011","journal-title":"Eur Respir J"},{"key":"2026022021141971100_bib25","doi-asserted-by":"crossref","first-page":"630","DOI":"10.1016\/S2213-2600(13)70182-6","article-title":"Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial","volume":"1","author":"Davies","year":"2013","journal-title":"Lancet Respir Med"},{"key":"2026022021141971100_bib26","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1136\/thoraxjnl-2017-210979","article-title":"Lung clearance index to monitor treatment response in pulmonary exacerbations in preschool children with cystic fibrosis","volume":"73","author":"Rayment","year":"2018","journal-title":"Thorax"},{"key":"2026022021141971100_bib27","doi-asserted-by":"crossref","first-page":"1216","DOI":"10.1164\/rccm.201610-2158OC","article-title":"Progression of lung disease in preschool patients with cystic fibrosis","volume":"195","author":"Stanojevic","year":"2017","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib28","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1016\/j.jcf.2019.11.006","article-title":"Integrating the multiple breath washout test into international multicentre trials","volume":"19","author":"Saunders","year":"2020","journal-title":"J Cyst Fibros"},{"key":"2026022021141971100_bib29","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":"Wielp\u00fctz","year":"2014","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib30","doi-asserted-by":"crossref","first-page":"S49","DOI":"10.1002\/ppul.23537","article-title":"Early detection and sensitive monitoring of CF lung disease: prospects of improved and safer imaging","volume":"51","author":"Mall","year":"2016","journal-title":"Pediatr Pulmonol"},{"key":"2026022021141971100_bib31","doi-asserted-by":"crossref","first-page":"1700244","DOI":"10.1183\/13993003.00244-2017","article-title":"Changes in magnetic resonance imaging scores and ventilation inhomogeneity in children with cystic fibrosis pulmonary exacerbations","volume":"50","author":"Grasemann","year":"2017","journal-title":"Eur Respir J"},{"key":"2026022021141971100_bib32","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1164\/rccm.201604-0893OC","article-title":"Comparison of lung clearance index and magnetic resonance imaging for assessment of lung disease in children with cystic fibrosis","volume":"195","author":"Stahl","year":"2017","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib33","doi-asserted-by":"crossref","first-page":"1238","DOI":"10.1164\/rccm.201807-1203OC","article-title":"Preventive inhalation of hypertonic saline in infants with cystic fibrosis (PRESIS): a randomized, double-blind, controlled study","volume":"199","author":"Stahl","year":"2019","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib34","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":"2026022021141971100_bib35","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1164\/rccm.202102-0278OC","article-title":"Magnetic resonance imaging detects progression of lung disease and impact of newborn screening in preschool children with cystic fibrosis","volume":"204","author":"Stahl","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib36","doi-asserted-by":"crossref","first-page":"1802188","DOI":"10.1183\/13993003.02188-2018","article-title":"Hyperpolarised 129Xe magnetic resonance imaging to monitor treatment response in children with cystic fibrosis","volume":"53","author":"Rayment","year":"2019","journal-title":"Eur Respir J"},{"key":"2026022021141971100_bib37","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":"2026022021141971100_bib38","doi-asserted-by":"crossref","first-page":"1028","DOI":"10.1002\/mrm.28457","article-title":"Comparison of single breath hyperpolarized 129Xe MRI with dynamic 19F MRI in cystic fibrosis lung disease","volume":"85","author":"McCallister","year":"2021","journal-title":"Magn Reson Med"},{"key":"2026022021141971100_bib39","doi-asserted-by":"crossref","first-page":"1701464","DOI":"10.1183\/13993003.01464-2017","article-title":"Novel magnetic resonance technique for functional imaging of cystic fibrosis lung disease","volume":"50","author":"Nyilas","year":"2017","journal-title":"Eur Respir J"},{"key":"2026022021141971100_bib40","doi-asserted-by":"crossref","first-page":"2003104","DOI":"10.1183\/13993003.03104-2020","article-title":"Free-breathing MRI for monitoring ventilation changes following antibiotic treatment of pulmonary exacerbations in paediatric cystic fibrosis","volume":"57","author":"Munidasa","year":"2021","journal-title":"Eur Respir J"},{"key":"2026022021141971100_bib41","doi-asserted-by":"crossref","first-page":"e209","DOI":"10.1016\/j.acra.2020.05.008","article-title":"Comparison of functional free-breathing pulmonary 1H and hyperpolarized 129Xe magnetic resonance imaging in pediatric cystic fibrosis","volume":"28","author":"Couch","year":"2021","journal-title":"Acad Radiol"},{"key":"2026022021141971100_bib42","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1016\/j.jcf.2021.03.016","article-title":"Peripheral lung effect of elexacaftor\/tezacaftor\/ivacaftor in adult cystic fibrosis","volume":"21","author":"Stylemans","year":"2022","journal-title":"J Cyst Fibros"},{"key":"2026022021141971100_bib43","doi-asserted-by":"crossref","first-page":"00408-2020","DOI":"10.1183\/23120541.00408-2020","article-title":"Multicentre feasibility of multiple-breath washout in preschool children with cystic fibrosis and other lung diseases","volume":"6","author":"Stahl","year":"2020","journal-title":"ERJ Open Res"},{"key":"2026022021141971100_bib44","doi-asserted-by":"crossref","first-page":"1148","DOI":"10.1152\/japplphysiol.00338.2021","article-title":"Correction of sensor crosstalk error in Exhalyzer D multiple-breath washout device significantly impacts outcomes in children with cystic fibrosis","volume":"131","author":"Wyler","year":"2021","journal-title":"J Appl Physiol"},{"key":"2026022021141971100_bib45","doi-asserted-by":"crossref","first-page":"2306","DOI":"10.1002\/mrm.26893","article-title":"Feasibility of quantitative regional ventilation and perfusion mapping with phase-resolved functional lung (PREFUL) MRI in healthy volunteers and COPD, CTEPH, and CF patients","volume":"79","author":"Voskrebenzev","year":"2018","journal-title":"Magn Reson Med"},{"key":"2026022021141971100_bib46","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1002\/jmri.27027","article-title":"Validation of automated Perfusion-Weighted Phase-Resolved Functional Lung (PREFUL)-MRI in patients with pulmonary diseases","volume":"52","author":"Behrendt","year":"2020","journal-title":"J Magn Reson Imaging"},{"key":"2026022021141971100_bib47","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":"2026022021141971100_bib48","author":"R Core Team","year":"2018"},{"key":"2026022021141971100_bib49","doi-asserted-by":"crossref","first-page":"1991","DOI":"10.1056\/NEJMoa0909825","article-title":"Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation","volume":"363","author":"Accurso","year":"2010","journal-title":"N Engl J Med"},{"key":"2026022021141971100_bib50","doi-asserted-by":"crossref","first-page":"1252","DOI":"10.1164\/rccm.201507-1271LE","article-title":"Intestinal current measurements detect activation of mutant CFTR in patients with cystic fibrosis with the G551D mutation treated with ivacaftor","volume":"192","author":"Graeber","year":"2015","journal-title":"Am J Respir Crit Care Med"},{"key":"2026022021141971100_bib51","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1016\/j.chest.2017.10.005","article-title":"CORK study in cystic fibrosis: sustained improvements in ultra-low-dose chest CT scores after CFTR modulation with ivacaftor","volume":"153","author":"Ronan","year":"2018","journal-title":"Chest"}],"container-title":["American Journal of Respiratory and Critical Care Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ajrccm\/article-pdf\/206\/3\/311\/66999946\/ajrccm_206_3_311.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ajrccm\/article-pdf\/206\/3\/311\/66999946\/ajrccm_206_3_311.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,21]],"date-time":"2026-02-21T02:14:30Z","timestamp":1771640070000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ajrccm\/article\/206\/3\/311\/8491598"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,1]]},"references-count":51,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2022,8,1]]},"published-print":{"date-parts":[[2022,8,1]]}},"URL":"https:\/\/doi.org\/10.1164\/rccm.202201-0219oc","relation":{},"ISSN":["1073-449X","1535-4970"],"issn-type":[{"value":"1073-449X","type":"print"},{"value":"1535-4970","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2022,8]]},"published":{"date-parts":[[2022,8,1]]},"assertion":[{"value":"2022-01-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-05-09","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-08-01","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}