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Πεδίο DCΤιμήΓλώσσα
dc.contributor.authorKouis, Panayiotis-
dc.contributor.authorGoutaki, Myrofora-
dc.contributor.authorHalbeisen, Florian S.-
dc.contributor.authorGioti, Ifigeneia-
dc.contributor.authorMiddleton, Nicos-
dc.contributor.authorAmirav, Israel-
dc.contributor.authorBarbato, Angelo-
dc.contributor.authorBehan, Laura-
dc.contributor.authorBoon, Mieke-
dc.contributor.authorEmiralioglu, Nagehan-
dc.contributor.authorHaarman, Eric G.-
dc.contributor.authorKaradag, Bulent-
dc.contributor.authorKoerner-Rettberg, Cordula-
dc.contributor.authorLazor, Romain-
dc.contributor.authorLoebinger, Michael R.-
dc.contributor.authorMaitre, Bernard-
dc.contributor.authorMazurek, Henryk-
dc.contributor.authorMorgan, Lucy-
dc.contributor.authorNielsen, Kim Gjerum-
dc.contributor.authorOmran, Heymut-
dc.contributor.authorÖzçelik, Ugur-
dc.contributor.authorPrice, Mareike-
dc.contributor.authorPogorzelski, Andrzej-
dc.contributor.authorSnijders, Deborah-
dc.contributor.authorThouvenin, Guillaume-
dc.contributor.authorWerner, Claudius-
dc.contributor.authorZivkovic, Zorica M.-
dc.contributor.authorKuehni, Claudia E.-
dc.contributor.authorYiallouros, Panayiotis K.-
dc.date.accessioned2020-07-24T05:54:37Z-
dc.date.available2020-07-24T05:54:37Z-
dc.date.issued2019-09-18-
dc.identifier.citationRespiratory Research, 2019, vol. 20, no. 1, articl. no. 212en_US
dc.identifier.issn1465993X-
dc.identifier.urihttps://hdl.handle.net/20.500.14279/18565-
dc.description.abstractBackground: Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients. Methods: In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally. Results: Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (-2.41 vs-1.35, p = 0.0001) and FEV1 z-scores (-2.79 vs-1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope:-0.037/year Vs-0.009/year, p = 0.047 and FEV1 z-score slope:-0.052/year Vs-0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function. Conclusions: Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.en_US
dc.formatpdfen_US
dc.language.isoenen_US
dc.relation.ispartofRespiratory Researchen_US
dc.rights© The Author(s)en_US
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/3.0/us/*
dc.subjectCiliary motility disorders (MeSH)en_US
dc.subjectKartagener syndrome (MeSH)en_US
dc.subjectLobectomyen_US
dc.titlePrevalence and course of disease after lung resection in primary ciliary dyskinesia: A cohort & nested case-control studyen_US
dc.typeArticleen_US
dc.collaborationUniversity of Cyprusen_US
dc.collaborationUniversity of Bernen_US
dc.collaborationCyprus University of Technologyen_US
dc.collaborationUniversity of Albertaen_US
dc.collaborationUniversity of Padovaen_US
dc.collaborationUniversity Hospital Southampton NHS Foundation Trusten_US
dc.collaborationUniversity of Southamptonen_US
dc.collaborationUniversity Hospital Gasthuisberg Leuvenen_US
dc.collaborationHacettepe Universityen_US
dc.collaborationVrije Universiteit Brusselen_US
dc.collaborationMarmara Universityen_US
dc.collaborationUniversity Children's Hospital of Ruhren_US
dc.collaborationUniversity Bochumen_US
dc.collaborationLausanne University Hospitalen_US
dc.collaborationNational Reference Centre for Rare Pulmonary Diseasesen_US
dc.collaborationRoyal Brompton and Harefield NHS Foundation Trusten_US
dc.collaborationUniversité Paris Est Créteilen_US
dc.collaborationInstytut Gruźlicy i ChoróbPłucen_US
dc.collaborationUniversity of Sydneyen_US
dc.collaborationCopenhagen University Hospitalen_US
dc.collaborationUniversity Hospital Muensteren_US
dc.collaborationHannover Medical Schoolen_US
dc.collaborationSorbonne Universitésen_US
dc.collaborationMedical Centre dr Dragisa Misovicen_US
dc.collaborationBusiness Academy in Novi Saden_US
dc.collaborationShakolas Educational Center of Clinical Medicineen_US
dc.collaborationTel Aviv Medical Centeren_US
dc.collaborationHelios Hospital Schwerinen_US
dc.subject.categoryClinical Medicineen_US
dc.journalsOpen Accessen_US
dc.countryCyprusen_US
dc.countrySwitzerlanden_US
dc.countryCanadaen_US
dc.countryItalyen_US
dc.countryUnited Kingdomen_US
dc.countryBelgiumen_US
dc.countryTurkeyen_US
dc.countryNetherlandsen_US
dc.countryGermanyen_US
dc.countryFranceen_US
dc.countryPolanden_US
dc.countryAustraliaen_US
dc.countryDenmarken_US
dc.countrySerbiaen_US
dc.countryIsraelen_US
dc.subject.fieldMedical and Health Sciencesen_US
dc.publicationPeer Revieweden_US
dc.identifier.doi10.1186/s12931-019-1183-yen_US
dc.identifier.pmid31533829-
dc.identifier.scopus2-s2.0-85072516815-
dc.identifier.urlhttps://api.elsevier.com/content/abstract/scopus_id/85072516815-
dc.relation.issue1en_US
dc.relation.volume20en_US
cut.common.academicyear2019-2020en_US
item.fulltextWith Fulltext-
item.languageiso639-1en-
item.grantfulltextopen-
item.openairecristypehttp://purl.org/coar/resource_type/c_6501-
item.cerifentitytypePublications-
item.openairetypearticle-
crisitem.journal.journalissn1465-993X-
crisitem.journal.publisherSpringer Nature-
crisitem.author.deptCyprus International Institute for Environmental and Public Health-
crisitem.author.deptDepartment of Nursing-
crisitem.author.deptCyprus International Institute for Environmental and Public Health-
crisitem.author.facultyFaculty of Health Sciences-
crisitem.author.facultyFaculty of Health Sciences-
crisitem.author.facultyFaculty of Health Sciences-
crisitem.author.orcid0000-0003-0511-5352-
crisitem.author.orcid0000-0001-6358-8591-
crisitem.author.orcid0000-0002-8339-9285-
crisitem.author.parentorgFaculty of Health Sciences-
crisitem.author.parentorgFaculty of Health Sciences-
crisitem.author.parentorgFaculty of Health Sciences-
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