{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T21:29:30Z","timestamp":1790803770876,"version":"4.1.0"},"reference-count":24,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T00:00:00Z","timestamp":1730419200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T00:00:00Z","timestamp":1730419200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.fr","cysticfibrosisjournal.com","clinicalkey.jp","clinicalkey.es","clinicalkey.com.au","clinicalkey.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Cystic Fibrosis"],"published-print":{"date-parts":[[2024,11]]},"DOI":"10.1016\/j.jcf.2024.04.013","type":"journal-article","created":{"date-parts":[[2024,5,9]],"date-time":"2024-05-09T13:04:31Z","timestamp":1715259871000},"page":"1072-1079","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":38,"title":["Heterogeneity in response to Elexacaftor\/Tezacaftor\/Ivacaftor in people with cystic fibrosis"],"prefix":"10.1016","volume":"23","author":[{"given":"Gianfranco","family":"Alicandro","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2315-5737","authenticated-orcid":false,"given":"Andrea","family":"Gramegna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-6891-0785","authenticated-orcid":false,"given":"Federica","family":"Bellino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sathya Calogero","family":"Sciarrabba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Lanfranchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4981-7076","authenticated-orcid":false,"given":"Martina","family":"Contarini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariangela","family":"Retucci","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Dacc\u00f2","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2285-9970","authenticated-orcid":false,"given":"Francesco","family":"Blasi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"10059","key":"10.1016\/j.jcf.2024.04.013_bib0001","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"},{"issue":"16","key":"10.1016\/j.jcf.2024.04.013_bib0002","doi-asserted-by":"crossref","first-page":"5882","DOI":"10.3390\/ijms21165882","article-title":"From Ivacaftor to triple combination: a systematic review of efficacy and safety of CFTR modulators in people with cystic fibrosis","volume":"21","author":"Gramegna","year":"2020","journal-title":"Int J Mol Sci"},{"issue":"1","key":"10.1016\/j.jcf.2024.04.013_bib0003","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.jcf.2022.10.002","article-title":"Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis","volume":"22","author":"Southern","year":"2023","journal-title":"J Cyst Fibros"},{"issue":"10212","key":"10.1016\/j.jcf.2024.04.013_bib0004","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1016\/S0140-6736(19)32597-8","article-title":"VX17-445-103 Trial Group. 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"},{"issue":"19","key":"10.1016\/j.jcf.2024.04.013_bib0005","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"VX17-445-102 Study Group. Elexacaftor-Tezacaftor-Ivacaftor for cystic fibrosis with a single Phe508del Allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N Engl J Med"},{"issue":"1","key":"10.1016\/j.jcf.2024.04.013_bib0006","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1186\/s12931-023-02451-0","article-title":"Heterogeneity of weight gain after initiation of Elexacaftor\/Tezacaftor\/Ivacaftor in people with cystic fibrosis","volume":"24","author":"Gramegna","year":"2023","journal-title":"Respir Res"},{"issue":"7","key":"10.1016\/j.jcf.2024.04.013_bib0007","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1007\/BF03344156","article-title":"Italian cross-sectional growth charts for height, weight and BMI (2 to 20 yr)","volume":"29","author":"Cacciari","year":"2006","journal-title":"J Endocrinol Invest"},{"issue":"6","key":"10.1016\/j.jcf.2024.04.013_bib0008","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1183\/09031936.00080312","article-title":"ERS Global Lung Function Initiative. Multi-ethnic reference values for spirometry for the 3-95-yr age range: the global lung function 2012 equations","volume":"40","author":"Quanjer","year":"2012","journal-title":"Eur Respir J"},{"issue":"2","key":"10.1016\/j.jcf.2024.04.013_bib0009","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/j.jcf.2016.12.008","article-title":"Chest imaging in cystic fibrosis studies: what counts, and can be counted?","volume":"16","author":"Szczesniak","year":"2017","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.jcf.2024.04.013_bib24","first-page":"1","article-title":"Relative Importance for Linear Regression in R: The Package relaimpo","volume":"17","author":"Gr\u00f6mping","year":"2007","journal-title":"J Stat Softw. Am. Stat. Assoc."},{"issue":"3","key":"10.1016\/j.jcf.2024.04.013_bib0010","doi-asserted-by":"crossref","DOI":"10.1001\/jamanetworkopen.2022.0740","article-title":"Association of body mass index with clinical outcomes in patients with cystic fibrosis: a systematic review and meta-analysis","volume":"5","author":"Nagy","year":"2022","journal-title":"JAMA Netw Open"},{"issue":"1","key":"10.1016\/j.jcf.2024.04.013_bib0011","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.jcf.2021.05.002","article-title":"Overweight and obesity in adults with cystic fibrosis: an Italian multicenter cohort study","volume":"21","author":"Gramegna","year":"2022","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.jcf.2024.04.013_bib0012","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1186\/s12916-015-0520-3","article-title":"Comparison of registered and published outcomes in randomized controlled trials: a systematic review","volume":"13","author":"Jones","year":"2015","journal-title":"BMC Med"},{"issue":"9","key":"10.1016\/j.jcf.2024.04.013_bib0013","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1164\/rccm.202308-1317OC","article-title":"Improvement in lung clearance index and chest computed tomography scores with Elexacaftor\/Tezacaftor\/Ivacaftor treatment in people with cystic fibrosis aged 12 years and older - the recover trial","volume":"208","author":"McNally","year":"2023","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.jcf.2024.04.013_bib0014","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.02153-2022","article-title":"Longitudinal effects of elexacaftor\/tezacaftor\/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis","volume":"62","author":"Schaupp","year":"2023","journal-title":"Eur Respir J"},{"issue":"9","key":"10.1016\/j.jcf.2024.04.013_bib0015","doi-asserted-by":"crossref","first-page":"1999","DOI":"10.3390\/jcm10091999","article-title":"Computed tomographic changes in patients with cystic fibrosis treated by combination therapy with lumacaftor and Ivacaftor","volume":"10","author":"Arnaud","year":"2021","journal-title":"J Clin Med"},{"key":"10.1016\/j.jcf.2024.04.013_bib0016","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.01344-2021","article-title":"French Cystic Fibrosis Reference Network Study Group. Using chest CT scan and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis","author":"Campredon","year":"2021","journal-title":"Eur Respir J"},{"issue":"6","key":"10.1016\/j.jcf.2024.04.013_bib0017","doi-asserted-by":"crossref","first-page":"1610","DOI":"10.1378\/chest.08-1190","article-title":"Determination of the minimal clinically important difference scores for the Cystic Fibrosis Questionnaire-Revised respiratory symptom scale in two populations of patients with cystic fibrosis and chronic Pseudomonas aeruginosa airway infection","volume":"135","author":"Quittner","year":"2009","journal-title":"Chest"},{"issue":"5","key":"10.1016\/j.jcf.2024.04.013_bib0018","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1164\/rccm.202108-1986OC","article-title":"PROMISE Study group. 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"},{"issue":"1","key":"10.1016\/j.jcf.2024.04.013_bib0019","doi-asserted-by":"crossref","first-page":"e7","DOI":"10.1016\/j.jcf.2020.05.004","article-title":"Females with cystic fibrosis have a larger decrease in sweat chloride in response to Lumacaftor\/Ivacaftor compared to males","volume":"20","author":"Aalbers","year":"2021","journal-title":"J Cyst Fibros"},{"issue":"8","key":"10.1016\/j.jcf.2024.04.013_bib0020","doi-asserted-by":"crossref","first-page":"996","DOI":"10.1164\/rccm.201909-1845LE","article-title":"Females with cystic fibrosis demonstrate a differential response profile to Ivacaftor compared with males","volume":"201","author":"Secunda","year":"2020","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.jcf.2024.04.013_bib0021","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.02437-2022","article-title":"Network study group. The French Compassionate Program of Elexacaftor-Tezacaftor-Ivacaftor in people with cystic fibrosis with advanced lung disease and no F508del CFTR variant","author":"Burgel","year":"2023","journal-title":"Eur Respir J"},{"issue":"6","key":"10.1016\/j.jcf.2024.04.013_bib0022","doi-asserted-by":"crossref","first-page":"458","DOI":"10.3390\/jpm11060458","article-title":"CFTR modulators: does one dose fit all?","volume":"11","author":"van der Meer","year":"2021","journal-title":"J Pers Med"},{"issue":"5","key":"10.1016\/j.jcf.2024.04.013_bib0023","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1097\/FTD.0b013e31826aabc3","article-title":"Safe and effective variability-a criterion for dose individualization","volume":"34","author":"Holford","year":"2012","journal-title":"Ther Drug Monit"}],"container-title":["Journal of Cystic Fibrosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199324000572?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199324000572?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,12,3]],"date-time":"2024-12-03T19:26:33Z","timestamp":1733253993000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1569199324000572"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11]]},"references-count":24,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2024,11]]}},"alternative-id":["S1569199324000572"],"URL":"https:\/\/doi.org\/10.1016\/j.jcf.2024.04.013","relation":{},"ISSN":["1569-1993"],"issn-type":[{"value":"1569-1993","type":"print"}],"subject":[],"published":{"date-parts":[[2024,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Heterogeneity in response to Elexacaftor\/Tezacaftor\/Ivacaftor in people with cystic fibrosis","name":"articletitle","label":"Article Title"},{"value":"Journal of Cystic Fibrosis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jcf.2024.04.013","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}