{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T02:31:49Z","timestamp":1791167509939,"version":"4.1.0"},"reference-count":43,"publisher":"European Respiratory Society (ERS)","issue":"2","funder":[{"DOI":"10.13039\/501100001711","name":"Schweizerischer Nationalfonds zur F\u00f6rderung der Wissenschaftlichen Forschung","doi-asserted-by":"publisher","award":["320030_173044"],"award-info":[{"award-number":["320030_173044"]}],"id":[{"id":"10.13039\/501100001711","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2018,6,22]]},"published-print":{"date-parts":[[2018,8]]},"abstract":"<jats:p>Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have been few and small.<\/jats:p><jats:p>This study compared lung function from spirometry of PCD patients to normal reference values and to published data from CF patients. We calculated z-scores and % predicted values for forced expiratory volume in 1\u2005s (FEV<jats:sub>1<\/jats:sub>) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD Cohort. We then assessed associations with age, sex, country, diagnostic certainty, organ laterality, body mass index and age at diagnosis in linear regression models. Lung function in PCD patients was reduced compared to reference values in both sexes and all age groups. Children aged 6\u20139\u2005years had the smallest impairment (FEV<jats:sub>1<\/jats:sub>z-score \u22120.84 (\u22121.03 to \u22120.65), FVC z-score \u22120.31 (\u22120.51 to \u22120.11)). Compared to CF patients, FEV<jats:sub>1<\/jats:sub>was similarly reduced in children (age 6\u20139\u2005years PCD 91% (88\u201393%); CF 90% (88\u201391%)), but less impaired in young adults (age 18\u201321\u2005years PCD 79% (76\u201382%); CF 66% (65\u201368%)). The results suggest that PCD affects lung function from early in life, which emphasises the importance of early standardised care for all patients.<\/jats:p>","DOI":"10.1183\/13993003.01040-2018","type":"journal-article","created":{"date-parts":[[2018,7,26]],"date-time":"2018-07-26T14:05:17Z","timestamp":1532613917000},"page":"1801040","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":94,"title":["Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study"],"prefix":"10.1183","volume":"52","author":[{"given":"Florian S.","family":"Halbeisen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8036-2092","authenticated-orcid":false,"given":"Myrofora","family":"Goutaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ben D.","family":"Spycher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6917-5285","authenticated-orcid":false,"given":"Israel","family":"Amirav","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Behan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mieke","family":"Boon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Hogg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen","family":"Casaulta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suzanne","family":"Crowley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric G.","family":"Haarman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bulent","family":"Karadag","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cordula","family":"Koerner-Rettberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael R.","family":"Loebinger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henryk","family":"Mazurek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucy","family":"Morgan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim G.","family":"Nielsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heymut","family":"Omran","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Santamaria","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolaus","family":"Schwerk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Thouvenin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Panayiotis","family":"Yiallouros","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8701-9975","authenticated-orcid":false,"given":"Jane\u00a0S.","family":"Lucas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5239-1571","authenticated-orcid":false,"given":"Philipp","family":"Latzin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8957-2002","authenticated-orcid":false,"given":"Claudia E.","family":"Kuehni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2018,7,26]]},"reference":[{"key":"2024102100322446000_52.2.1801040.1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00176608"},{"key":"2024102100322446000_52.2.1801040.2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00736-2016"},{"key":"2024102100322446000_52.2.1801040.3","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02018-2015"},{"key":"2024102100322446000_52.2.1801040.4","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00001010"},{"key":"2024102100322446000_52.2.1801040.5","doi-asserted-by":"crossref","unstructured":"Lucas JS , Walker WT , Kuehni CE , et\u00a0al. 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