{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,8]],"date-time":"2026-02-08T17:45:23Z","timestamp":1770572723765,"version":"3.49.0"},"reference-count":13,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2023,3,20]],"date-time":"2023-03-20T00:00:00Z","timestamp":1679270400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Respiratory J"],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Ivacaftor has shown to be effective in patients with cystic fibrosis (CF) with a G551D mutation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>This work aims to evaluate ivacaftor's effectiveness and safety in the real world, over 5\u00a0years, in the West of Scotland CF population.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We evaluated ivacaftor's effect on pulmonary function, body mass index (BMI), hospital bed occupancy, and adverse effects in patients \u22656\u00a0years with at least one G551D mutation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Statistically significant increases from baseline were observed in mean per cent predicted forced expiratory volume in 1\u00a0s (FEV<jats:sub>1<\/jats:sub>) at year 1 (which was maintained at years 2 and 5) and BMI over 5\u00a0years in our adolescent\/adult cohort. Improvements were observed in per cent predicted FEV<jats:sub>1<\/jats:sub> within the paediatric cohort with a suggestion of a plateau effect. The increase in paediatric BMI <jats:italic>z<\/jats:italic>\u2010score was nonstatistically significant. There was a reduction in the number of pulmonary exacerbations requiring intravenous antibiotics and hospital bed occupancy. Ivacaftor was well tolerated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Ivacaftor was effective in our population.<\/jats:p><\/jats:sec>","DOI":"10.1111\/crj.13602","type":"journal-article","created":{"date-parts":[[2023,3,20]],"date-time":"2023-03-20T10:10:37Z","timestamp":1679307037000},"page":"473-477","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Ivacaftor: Five\u2010year outcomes in the West of Scotland cystic fibrosis population"],"prefix":"10.1111","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2218-407X","authenticated-orcid":false,"given":"Yasmin","family":"Al\u2010Din","sequence":"first","affiliation":[{"name":"Department of Pharmacy Queen Elizabeth University Hospital, NHS Greater Glasgow and Clyde  Glasgow UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carol","family":"Dryden","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University Hospital Wishaw, NHS Lanarkshire  Wishaw UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gordon","family":"MacGregor","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Queen Elizabeth University Hospital, NHS Greater Glasgow and Clyde  Glasgow UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Young","sequence":"additional","affiliation":[{"name":"Department of Mathematics and Statistics University of Strathclyde  Glasgow UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristina","family":"Coelho","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Glasgow Royal Infirmary, NHS Greater Glasgow and Clyde  Glasgow UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,3,20]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.k1783"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201301\u20100153OC"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1105185"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(14)70218-8"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.73.1.25"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.73.1.17"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild\u20102015\u2010310420"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.07.001"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1569\u20101993(19)30185\u20107"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1569\u20101993(19)30545\u20104"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1080\/13696998.2016.1178125"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2015.05.009"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.05.015"}],"container-title":["The Clinical Respiratory Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/crj.13602","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/crj.13602","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/crj.13602","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,19]],"date-time":"2023-08-19T14:22:10Z","timestamp":1692454930000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/crj.13602"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,20]]},"references-count":13,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2023,5]]}},"alternative-id":["10.1111\/crj.13602"],"URL":"https:\/\/doi.org\/10.1111\/crj.13602","archive":["Portico"],"relation":{},"ISSN":["1752-6981","1752-699X"],"issn-type":[{"value":"1752-6981","type":"print"},{"value":"1752-699X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,3,20]]},"assertion":[{"value":"2022-09-08","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-02-27","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-03-20","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}