{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T04:06:28Z","timestamp":1772165188963,"version":"3.50.1"},"reference-count":22,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:00Z","timestamp":1735689600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:00Z","timestamp":1735689600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Ther Adv Respir Dis"],"published-print":{"date-parts":[[2025,1]]},"DOI":"10.1177\/17534666251329792","type":"journal-article","created":{"date-parts":[[2025,4,2]],"date-time":"2025-04-02T02:36:00Z","timestamp":1743561360000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Cystic fibrosis: a model for research and management of respiratory diseases"],"prefix":"10.1177","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2314-0818","authenticated-orcid":false,"given":"Almudena","family":"Felipe Montiel","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine (Adult Cystic Fibrosis Unit), Vall d\u2019Hebron University Hospital, Vall d\u2019Hebron Research Institute (VHIR). Universitat Aut\u00f2noma de Barcelona (UAB), Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"\u00c1lvarez Fern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine (Adult Cystic Fibrosis Unit), Vall d\u2019Hebron University Hospital, Vall d\u2019Hebron Research Institute (VHIR), Universitat Aut\u00f2noma de Barcelona (UAB), Barcelona, Spain"},{"name":"CIBER de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alvar","family":"Agust\u00ed","sequence":"additional","affiliation":[{"name":"CIBER de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Madrid, Spain"},{"name":"University of Barcelona, Pulmonary Service, Respiratory Institute, Clinic Barcelona, FCRB-IDIBAPS Barcelona, Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4058-5109","authenticated-orcid":false,"given":"Eva","family":"Polverino","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, (Adult Cystic Fibrosis Unit), Vall d\u2019Hebron University Hospital, Vall d\u2019Hebron Research Institute (VHIR). Universitat Aut\u00f2noma de Barcelona (UAB), Pg. Vall d\u2019Hebron 119-129, Barcelona 08035, Spain"},{"name":"CIBER de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2025,4]]},"reference":[{"key":"e_1_3_4_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra2216474"},{"key":"e_1_3_4_3_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200505-840OE"},{"key":"e_1_3_4_4_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201009-1478CI"},{"key":"e_1_3_4_5_2","unstructured":"Zolin A Adamoli A Bakkeheim E et al. ECFSPR annual report 2022 ecfs.eu\/projects\/ecfs-patient-registry\/annual-reports (2024 accessed 24 July 2024)."},{"key":"e_1_3_4_6_2","doi-asserted-by":"crossref","DOI":"10.1016\/j.lpm.2017.05.011","article-title":"Current and emerging comorbidities in cystic fibrosis","volume":"46","author":"Ronan NJ","year":"2017","unstructured":"Ronan NJ, Elborn JS, Plant BJ. Current and emerging comorbidities in cystic fibrosis. Press Medicale 2017; 46: e125\u2013e138.","journal-title":"Press Medicale"},{"issue":"4","key":"e_1_3_4_7_2","doi-asserted-by":"crossref","first-page":"2300110","DOI":"10.1183\/13993003.00110-2023","article-title":"Theratyping cystic fibrosis patients to guide elexacaftor\/tezacaftor\/ivacaftor out-of-label prescription","volume":"62","author":"Dreano E","year":"2023","unstructured":"Dreano E, Burgel PR, Hatton A, et al. Theratyping cystic fibrosis patients to guide elexacaftor\/tezacaftor\/ivacaftor out-of-label prescription. Eur Respir J 2023; 62(4): 2300110.","journal-title":"Eur Respir J"},{"key":"e_1_3_4_8_2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3389\/fcell.2022.899368","article-title":"Lung organoids \u2013 the ultimate tool to dissect pulmonary diseases?","volume":"10","author":"Bos\u00e1kov\u00e1 V","year":"2022","unstructured":"Bos\u00e1kov\u00e1 V, De Zuani M, Sl\u00e1dkov\u00e1 L, et al. Lung organoids \u2013 the ultimate tool to dissect pulmonary diseases?. Front Cell Dev Biol 2022; 10: 1\u201319.","journal-title":"Front Cell Dev Biol"},{"key":"e_1_3_4_9_2","first-page":"1","article-title":"Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond","volume":"61","author":"Mall MA","year":"2023","unstructured":"Mall MA, Criner GJ, Miravitlles M, et al. Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond. Eur Respir J 2023; 61: 1\u201319.","journal-title":"Eur Respir J"},{"key":"e_1_3_4_10_2","doi-asserted-by":"crossref","first-page":"2399","DOI":"10.2147\/COPD.S257474","article-title":"Efficacy and safety of the cftr potentiator icenticaftor (Qbw251) in copd: results from a phase 2 randomized trial","volume":"15","author":"Rowe SM","year":"2020","unstructured":"Rowe SM, Jones I, Dransfield MT, et al. Efficacy and safety of the cftr potentiator icenticaftor (Qbw251) in copd: results from a phase 2 randomized trial. Int J COPD 2020; 15: 2399\u20132409.","journal-title":"Int J COPD"},{"key":"e_1_3_4_11_2","article-title":"Evaluating elexacaftor\/tezacaftor\/ivacaftor (ETI; TrikaftaTM) for treatment of patients with non-cystic fibrosis bronchiectasis (NCFB): a clinical study protocol","author":"Swenson CE","year":"2024","unstructured":"Swenson CE, Hunt WR, Manfredi C, et al. Evaluating elexacaftor\/tezacaftor\/ivacaftor (ETI; TrikaftaTM) for treatment of patients with non-cystic fibrosis bronchiectasis (NCFB): a clinical study protocol. medRxiv 2024; 2024.07.19.24310604.","journal-title":"medRxiv"},{"key":"e_1_3_4_12_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.54.3.358"},{"key":"e_1_3_4_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.arbres.2021.03.009"},{"issue":"6","key":"e_1_3_4_14_2","first-page":"1140","article-title":"A comparison between two pathophysiologically different yet microbiologically similar lung diseases: cystic fibrosis and chronic obstructive pulmonary disease","volume":"11","author":"Daniel EF","year":"2019","unstructured":"Daniel EF, Cameron TM, Warunya P, et al. A comparison between two pathophysiologically different yet microbiologically similar lung diseases: cystic fibrosis and chronic obstructive pulmonary disease. Metallomics 2019; 11(6): 1140\u20131153.","journal-title":"Metallomics"},{"key":"e_1_3_4_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2021.106572"},{"key":"e_1_3_4_16_2","article-title":"The in-between: time to talk about bronchiectasis in adolescents and their transition to adult care","author":"Traversi L","year":"2024","unstructured":"Traversi L, Garriga-Grimau L, Moreno-Gald\u00f3 A, et al. The in-between: time to talk about bronchiectasis in adolescents and their transition to adult care. Arch Bronconeumol 2024: S0300\u20132896(24)00359\u20134. doi: 10.1016\/j.arbres.2024.09.001.","journal-title":"Arch Bronconeumol"},{"key":"e_1_3_4_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2023.01.010"},{"issue":"1","key":"e_1_3_4_18_2","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1186\/s12931-022-02221-4","article-title":"A review of the challenges, learnings and future directions of home handheld spirometry in interstitial lung disease","volume":"23","author":"Maher TM","year":"2022","unstructured":"Maher TM, Schiffman C, Kreuter M, et al. A review of the challenges, learnings and future directions of home handheld spirometry in interstitial lung disease. Respir Res 2022; 23(1): 307.","journal-title":"Respir Res"},{"key":"e_1_3_4_19_2","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1007\/s00408-018-0086-x","article-title":"Primary ciliary dyskinesia due to microtubular defects is associated with worse lung clearance index","volume":"196","author":"Irving S","year":"2018","unstructured":"Irving S, Dixon M, Fassad MR, et al. Primary ciliary dyskinesia due to microtubular defects is associated with worse lung clearance index. Lung 2018; 196: 231\u2013238.","journal-title":"Lung"},{"key":"e_1_3_4_20_2","doi-asserted-by":"crossref","first-page":"1210","DOI":"10.1183\/09031936.00085713","article-title":"Double tracer gas single-breath washout: reproducibility in healthy subjects and copd","volume":"44","author":"Husemann K","year":"2014","unstructured":"Husemann K, Berg N, Engel J, et al. Double tracer gas single-breath washout: reproducibility in healthy subjects and copd. Eur Respir J 2014; 44: 1210\u20131222.","journal-title":"Eur Respir J"},{"key":"e_1_3_4_21_2","doi-asserted-by":"crossref","first-page":"1254","DOI":"10.1016\/j.rmed.2014.07.003","article-title":"Clinical application of inert gas multiple breath washout in children and adolescents with asthma","volume":"108","author":"Zwitserloot A","year":"2014","unstructured":"Zwitserloot A, Fuchs SI, M\u00fcller C, et al. Clinical application of inert gas multiple breath washout in children and adolescents with asthma. Respir Med 2014; 108: 1254\u20131259.","journal-title":"Respir Med"},{"issue":"1","key":"e_1_3_4_22_2","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1186\/1465-9921-15-59","article-title":"Lung clearance index in adults with non-cystic fibrosis bronchiectasis","volume":"15","author":"Gonem S","year":"2014","unstructured":"Gonem S, Scadding A, Soares M, et al. Lung clearance index in adults with non-cystic fibrosis bronchiectasis. Respir Res 2014; 15(1): 59.","journal-title":"Respir Res"},{"key":"e_1_3_4_23_2","doi-asserted-by":"publisher","DOI":"10.2217\/frd-2022-0003"}],"container-title":["Therapeutic Advances in Respiratory Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/17534666251329792","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/17534666251329792","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/17534666251329792","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,6]],"date-time":"2026-01-06T08:20:10Z","timestamp":1767687610000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/17534666251329792"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1]]},"references-count":22,"alternative-id":["10.1177\/17534666251329792"],"URL":"https:\/\/doi.org\/10.1177\/17534666251329792","relation":{"has-review":[{"id-type":"doi","id":"10.1177\/17534666251329792\/v3\/response1","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/17534666251329792\/v2\/review2","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/17534666251329792\/v1\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/17534666251329792\/v2\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/17534666251329792\/v1\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/17534666251329792\/v2\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/17534666251329792\/v2\/response1","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/17534666251329792\/v1\/review2","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/17534666251329792\/v3\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/17534666251329792\/v1\/review3","asserted-by":"object"}]},"ISSN":["1753-4666","1753-4666"],"issn-type":[{"value":"1753-4666","type":"print"},{"value":"1753-4666","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1]]},"article-number":"17534666251329792"}}