{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T07:11:26Z","timestamp":1790665886825,"version":"4.1.0"},"reference-count":15,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2023,5,1]],"date-time":"2023-05-01T00:00:00Z","timestamp":1682899200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,5,1]],"date-time":"2023-05-01T00:00:00Z","timestamp":1682899200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,5,1]],"date-time":"2025-05-01T00:00:00Z","timestamp":1746057600000},"content-version":"vor","delay-in-days":731,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100011022","name":"Vertex Pharmaceuticals Incorporated","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011022","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","cysticfibrosisjournal.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Cystic Fibrosis"],"published-print":{"date-parts":[[2023,5]]},"DOI":"10.1016\/j.jcf.2022.12.006","type":"journal-article","created":{"date-parts":[[2022,12,27]],"date-time":"2022-12-27T07:24:37Z","timestamp":1672125877000},"page":"464-470","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":8,"title":["Long-term tezacaftor\/ivacaftor safety and efficacy in people with cystic fibrosis and an F508del-CFTR mutation: 96-week, open-label extension of the EXTEND trial"],"prefix":"10.1016","volume":"22","author":[{"given":"Patrick A.","family":"Flume","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. Scott","family":"Harris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hildegarde","family":"Paz-Diaz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil","family":"Ahluwalia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Higgins","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Campbell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Indrias","family":"Berhane","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judy L.","family":"Shih","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory","family":"Sawicki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"1","key":"10.1016\/j.jcf.2022.12.006_bib0001","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"},{"issue":"8","key":"10.1016\/j.jcf.2022.12.006_bib0002","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1016\/S2213-2600(16)00023-0","article-title":"Progress in therapies for cystic fibrosis","volume":"4","author":"De Boeck","year":"2016","journal-title":"Lancet Respir Med"},{"issue":"2","key":"10.1016\/j.jcf.2022.12.006_bib0003","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1016\/S2213-2600(12)70057-7","article-title":"A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect","volume":"1","author":"Boyle","year":"2013","journal-title":"Lancet Respir Med"},{"issue":"2","key":"10.1016\/j.jcf.2022.12.006_bib0004","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1002\/ppul.24215","article-title":"Patients with cystic fibrosis having a residual function mutation: data from the Italian registry","volume":"54","author":"Salvatore","year":"2019","journal-title":"Pediatr Pulmonol"},{"issue":"3","key":"10.1016\/j.jcf.2022.12.006_bib0005","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1016\/j.jcf.2008.03.009","article-title":"Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice","volume":"7","author":"Castellani","year":"2008","journal-title":"J Cyst Fibros"},{"issue":"21","key":"10.1016\/j.jcf.2022.12.006_bib0006","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"},{"issue":"21","key":"10.1016\/j.jcf.2022.12.006_bib0007","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"},{"issue":"7","key":"10.1016\/j.jcf.2022.12.006_bib0008","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"},{"issue":"3","key":"10.1016\/j.jcf.2022.12.006_bib0009","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"},{"issue":"2","key":"10.1016\/j.jcf.2022.12.006_bib0010","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"},{"issue":"11","key":"10.1016\/j.jcf.2022.12.006_bib0011","doi-asserted-by":"crossref","first-page":"902","DOI":"10.1016\/S2213-2600(14)70218-8","article-title":"Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST)","volume":"2","author":"McKone","year":"2014","journal-title":"Lancet Respir Med"},{"issue":"2","key":"10.1016\/j.jcf.2022.12.006_bib0012","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":"10.1016\/j.jcf.2022.12.006_bib0013","series-title":"FDA approves new breakthrough therapy for cystic fibrosis","year":"2019"},{"issue":"3","key":"10.1016\/j.jcf.2022.12.006_bib0014","first-page":"192","article-title":"Elexacaftor-tezacaftor-ivacaftor: the first triple-combination cystic fibrosis transmembrane conductance regulator modulating therapy","volume":"25","author":"Ridley","year":"2020","journal-title":"J Pediatr Pharmacol Ther"},{"issue":"10212","key":"10.1016\/j.jcf.2022.12.006_bib0015","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"}],"container-title":["Journal of Cystic Fibrosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199322014266?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199322014266?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T21:49:16Z","timestamp":1761601756000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1569199322014266"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5]]},"references-count":15,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,5]]}},"alternative-id":["S1569199322014266"],"URL":"https:\/\/doi.org\/10.1016\/j.jcf.2022.12.006","relation":{},"ISSN":["1569-1993"],"issn-type":[{"value":"1569-1993","type":"print"}],"subject":[],"published":{"date-parts":[[2023,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Long-term tezacaftor\/ivacaftor safety and efficacy in people with cystic fibrosis and an F508del-CFTR mutation: 96-week, open-label extension of the EXTEND trial","name":"articletitle","label":"Article Title"},{"value":"Journal of Cystic Fibrosis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jcf.2022.12.006","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2022 European Cystic Fibrosis Society. Published by Elsevier B.V.","name":"copyright","label":"Copyright"}]}}