{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,31]],"date-time":"2026-01-31T15:39:17Z","timestamp":1769873957856,"version":"3.49.0"},"reference-count":14,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2026,1,31]],"date-time":"2026-01-31T00:00:00Z","timestamp":1769817600000},"content-version":"vor","delay-in-days":122,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["www.atsjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,10,1]]},"DOI":"10.1164\/rccm.202508-1983ed","type":"journal-article","created":{"date-parts":[[2025,9,8]],"date-time":"2025-09-08T18:44:46Z","timestamp":1757357086000},"page":"1731-1732","update-policy":"https:\/\/doi.org\/10.1164\/crossmarkpolicies","source":"Crossref","is-referenced-by-count":0,"title":["Cystic Fibrosis: Huge Gains, but Still a Work in Progress"],"prefix":"10.1093","volume":"211","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6756-9822","authenticated-orcid":false,"given":"Andrew","family":"Bush","sequence":"first","affiliation":[{"name":"National Heart and Lung Institute Imperial College and Imperial Centre for Paediatrics and Child Health London, United Kingdom"},{"name":"Royal Brompton Hospital London, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bonnie W.","family":"Ramsey","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Washington School of Medicine Seattle, Washington"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2025,10,1]]},"reference":[{"key":"2026013020471029100_bib1","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1164\/rccm.202008-3177OC","article-title":"Ivacaftor in infants aged 4 to &lt;12 months with cystic fibrosis and a gating mutation. Results of a two-part phase 3 clinical trial","volume":"203","author":"Davies","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"2026013020471029100_bib2","doi-asserted-by":"crossref","first-page":"e71222","DOI":"10.1002\/ppul.71222","article-title":"Cystic fibrosis year in review 2024","volume":"60","author":"Savant","year":"2025","journal-title":"Pediatr Pulmonol"},{"key":"2026013020471029100_bib3","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1164\/rccm.202303-0380LE","article-title":"Raised intracranial pressure in three children with cystic fibrosis receiving elexacaftor-tezacaftor-ivacaftor modulator therapy","volume":"208","author":"Southern","year":"2023","journal-title":"Am J Respir Crit Care Med"},{"key":"2026013020471029100_bib4","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1002\/ppul.20138","article-title":"Renal impairment in cystic fibrosis patients due to repeated intravenous aminoglycoside use","volume":"39","author":"Al-Aloul","year":"2005","journal-title":"Pediatr Pulmonol"},{"key":"2026013020471029100_bib5","doi-asserted-by":"crossref","first-page":"1556","DOI":"10.1164\/rccm.202504-0841VP","article-title":"Is your placebo really needed?","volume":"211","author":"Bush","year":"2025","journal-title":"Am J Respir Crit Care Med"},{"key":"2026013020471029100_bib6","doi-asserted-by":"crossref","first-page":"1501","DOI":"10.1164\/rccm.202310-1831LE","article-title":"Human airway and alveolar organoids from BAL fluid","volume":"209","author":"Liu","year":"2024","journal-title":"Am J Respir Crit Care Med"},{"key":"2026013020471029100_bib7","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1186\/s13023-022-02350-5","article-title":"The impact of FDA and EMA regulatory decision-making process on the access to CFTR modulators for the treatment of cystic fibrosis","volume":"17","author":"Costa","year":"2022","journal-title":"Orphanet J Rare Dis"},{"key":"2026013020471029100_bib8","doi-asserted-by":"crossref","first-page":"2400156","DOI":"10.1183\/13993003.00156-2024","article-title":"Precision medicine in cystic fibrosis: predictive role of forskolin-induced swelling assay","volume":"63","author":"Furstova","year":"2024","journal-title":"Eur Respir J"},{"key":"2026013020471029100_bib9","doi-asserted-by":"crossref","first-page":"2202437","DOI":"10.1183\/13993003.02437-2022","article-title":"The French compassionate programme of elexacaftor\/tezacaftor\/ivacaftor in people with cystic fibrosis with advanced lung disease and no F508del CFTR variant","volume":"61","author":"Burgel","year":"2023","journal-title":"Eur Respir J"},{"key":"2026013020471029100_bib10","doi-asserted-by":"crossref","first-page":"1231","DOI":"10.1164\/rccm.202101-0090LE","article-title":"Elexacaftor\/tezacaftor\/ivacaftor improved clinical outcomes in a patient with N1303K-CFTR based on in vitro experimental evidence","volume":"204","author":"Huang","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"2026013020471029100_bib11","doi-asserted-by":"crossref","first-page":"947","DOI":"10.1016\/S2213-2600(24)00205-4","article-title":"Evaluation of elexacaftor-tezacaftor-ivacaftor treatment in individuals with cystic fibrosis and CFTR(N1303K) in the USA: a prospective, multicentre, open-label, single-arm trial","volume":"12","author":"Solomon","year":"2024","journal-title":"Lancet Respir Med"},{"key":"2026013020471029100_bib12","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1164\/rccm.202407-1302OC","article-title":"Ivacaftor for chronic obstructive pulmonary disease: results from a phase 2, randomized controlled trial","volume":"211","author":"Vijaykumar","year":"2024","journal-title":"Am J Respir Crit Care Med"},{"key":"2026013020471029100_bib13","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1164\/rccm.202409-1824OC","article-title":"Infection by clonally related Mycobacterium abscessus isolates: the role of drinking water","volume":"211","author":"Thomson","year":"2025","journal-title":"Am J Respir Crit Care Med"},{"key":"2026013020471029100_bib14","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1164\/rccm.202503-0579ED","article-title":"Water, water, everywhere, nor any drop to drink: beware of mycobacteria [editorial]","volume":"211","author":"Qvist","year":"2025","journal-title":"Am J Respir Crit Care Med"}],"container-title":["American Journal of Respiratory and Critical Care Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ajrccm\/article-pdf\/211\/10\/1731\/66621426\/ajrccm_211_10_1731.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ajrccm\/article-pdf\/211\/10\/1731\/66621426\/ajrccm_211_10_1731.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,31]],"date-time":"2026-01-31T01:47:21Z","timestamp":1769824041000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ajrccm\/article\/211\/10\/1731\/8444214"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,1]]},"references-count":14,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2025,10,1]]},"published-print":{"date-parts":[[2025,10,1]]}},"URL":"https:\/\/doi.org\/10.1164\/rccm.202508-1983ed","relation":{},"ISSN":["1073-449X","1535-4970"],"issn-type":[{"value":"1073-449X","type":"print"},{"value":"1535-4970","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2025,10]]},"published":{"date-parts":[[2025,10,1]]},"assertion":[{"value":"2025-09-26","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}