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Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study

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dc.contributor.authorKARADAĞ, BÜLENT TANER
dc.contributor.authorsHalbeisen, Florian S.; Goutaki, Myrofora; Spycher, Ben D.; Amirav, Israel; Behan, Laura; Boon, Mieke; Hogg, Claire; Casaulta, Carmen; Crowley, Suzanne; Haarman, Eric G.; Karadag, Bulent; Koerner-Rettberg, Cordula; Loebinger, Michael R.; Mazurek, Henryk; Morgan, Lucy; Nielsen, Kim G.; Omran, Heymut; Santamaria, Francesca; Schwerk, Nicolaus; Thouvenin, Guillaume; Yiallouros, Panayiotis; Lucas, Jane S.; Latzin, Philipp; Kuehni, Claudia E.
dc.date.accessioned2022-03-14T08:41:37Z
dc.date.available2022-03-14T08:41:37Z
dc.date.issued2018-08
dc.description.abstractPrimary 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. 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 s (FEV1) 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 6-9 years had the smallest impairment (FEVI z-score -0.84 (-1.03 to -0.65), FVC z-score -0.31 (-0.51 to -0.11)). Compared to CF patients, ITV' was similarly reduced in children (age 6-9 years PCD 91% (88-93%); CF 90% (88-91%)), but less impaired in young adults (age 18 21 years PCD 79% (76-82%); CF 66% (65-68%)). The results suggest that PCD affects lung function from early in life, which emphasises the importance of early standardised care for all patients.
dc.identifier.doi10.1183/13993003.01040-2018
dc.identifier.eissn1399-3003
dc.identifier.issn0903-1936
dc.identifier.pubmed30049738
dc.identifier.urihttps://hdl.handle.net/11424/242140
dc.identifier.wosWOS:000448078200017
dc.language.isoeng
dc.publisherEUROPEAN RESPIRATORY SOC JOURNALS LTD
dc.relation.ispartofEUROPEAN RESPIRATORY JOURNAL
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectCYSTIC-FIBROSIS
dc.subjectCLINICAL-FEATURES
dc.subjectEUROPEAN CHILDREN
dc.subjectUK
dc.subjectPOPULATION
dc.subjectSPIROMETRY
dc.subjectEQUATIONS
dc.subjectDISEASE
dc.subjectINDEX
dc.subjectAGE
dc.titleLung function in patients with primary ciliary dyskinesia: an iPCD Cohort study
dc.typearticle
dspace.entity.typePublication
local.avesis.idfb1078aa-2299-4bc4-ab36-6bba987b538a
local.import.packageSS16
local.indexed.atWOS
local.indexed.atSCOPUS
local.indexed.atPUBMED
local.journal.articlenumber1801040
local.journal.numberofpages11
local.journal.quartileQ1
oaire.citation.issue2
oaire.citation.titleEUROPEAN RESPIRATORY JOURNAL
oaire.citation.volume52
relation.isAuthorOfPublication218b489d-97ab-49dd-9db6-0c2a732f1999
relation.isAuthorOfPublication.latestForDiscovery218b489d-97ab-49dd-9db6-0c2a732f1999

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