The international primary ciliary dyskinesia cohort (iPCD Cohort): methods and first results
Identifieur interne : 000193 ( Main/Exploration ); précédent : 000192; suivant : 000194The international primary ciliary dyskinesia cohort (iPCD Cohort): methods and first results
Auteurs : Myrofora Goutaki [Suisse] ; Elisabeth Maurer [Suisse] ; Florian S. Halbeisen [Suisse] ; Israel Amirav [Israël] ; Angelo Barbato [Italie] ; Laura Behan [Royaume-Uni] ; Mieke Boon [Belgique] ; Carmen Casaulta [Suisse] ; Annick Clement [France] ; Suzanne Crowley [Norvège] ; Eric Haarman [Pays-Bas] ; Claire Hogg [Royaume-Uni] ; Bulent Karadag [Turquie] ; Cordula Koerner-Rettberg [Allemagne] ; Margaret W. Leigh [États-Unis] ; Michael R. Loebinger [Royaume-Uni] ; Henryk Mazurek [Pologne] ; Lucy Morgan [Australie] ; Kim G. Nielsen [Danemark] ; Heymut Omran [Allemagne] ; Nicolaus Schwerk [Allemagne] ; Sergio Scigliano [Argentine] ; Claudius Werner [Allemagne] ; Panayiotis Yiallouros [Chypre (pays)] ; Zorica Zivkovic [Serbie] ; Jane S. Lucas [Royaume-Uni] ; Claudia E. Kuehni [Suisse]Source :
- The European Respiratory Journal [ 0903-1936 ] ; 2017.
Abstract
Data on primary ciliary dyskinesia (PCD) epidemiology is scarce and published studies are characterised by low numbers. In the framework of the European Union project BESTCILIA we aimed to combine all available datasets in a retrospective international PCD cohort (iPCD Cohort).
We identified eligible datasets by performing a systematic review of published studies containing clinical information on PCD, and by contacting members of past and current European Respiratory Society Task Forces on PCD. We compared the contents of the datasets, clarified definitions and pooled them in a standardised format.
As of April 2016 the iPCD Cohort includes data on 3013 patients from 18 countries. It includes data on diagnostic evaluations, symptoms, lung function, growth and treatments. Longitudinal data are currently available for 542 patients. The extent of clinical details per patient varies between centres. More than 50% of patients have a definite PCD diagnosis based on recent guidelines. Children aged 10–19 years are the largest age group, followed by younger children (≤9 years) and young adults (20–29 years).
This is the largest observational PCD dataset available to date. It will allow us to answer pertinent questions on clinical phenotype, disease severity, prognosis and effect of treatments, and to investigate genotype–phenotype correlations.
Url:
DOI: 10.1183/13993003.01181-2016
PubMed: 28052956
PubMed Central: 5298195
Affiliations:
- Allemagne, Argentine, Australie, Belgique, Chypre (pays), Danemark, France, Israël, Italie, Norvège, Pays-Bas, Pologne, Royaume-Uni, Serbie, Suisse, Turquie, États-Unis
- Angleterre, Basse-Saxe, Canton de Berne, Caroline du Nord, Grand Londres, Hollande-Septentrionale, Hovedstaden, Nouvelle-Galles du Sud, Île-de-France, Østlandet
- Amsterdam, Berne, Copenhague, Hanovre, Londres, Oslo, Paris, Sydney
- Université de Berne, Université de Sydney
Links toward previous steps (curation, corpus...)
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- to stream Pmc, to step Checkpoint: 000038
- to stream Ncbi, to step Merge: 004121
- to stream Ncbi, to step Curation: 004121
- to stream Ncbi, to step Checkpoint: 004121
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Le document en format XML
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Leigh</name><affiliation wicri:level="2"><nlm:aff id="af13">Dept of Pediatrics, UNC School of Medicine, Chapel Hill, NC, USA</nlm:aff><country xml:lang="fr">États-Unis</country><wicri:regionArea>Dept of Pediatrics, UNC School of Medicine, Chapel Hill, NC</wicri:regionArea><placeName><region type="state">Caroline du Nord</region></placeName></affiliation></author><author><name sortKey="Loebinger, Michael R" sort="Loebinger, Michael R" uniqKey="Loebinger M" first="Michael R." last="Loebinger">Michael R. 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Nielsen</name><affiliation wicri:level="3"><nlm:aff id="af17">Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark</nlm:aff><country xml:lang="fr">Danemark</country><wicri:regionArea>Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen</wicri:regionArea><placeName><settlement type="city">Copenhague</settlement><region type="région" nuts="2">Hovedstaden</region></placeName></affiliation></author><author><name sortKey="Omran, Heymut" sort="Omran, Heymut" uniqKey="Omran H" first="Heymut" last="Omran">Heymut Omran</name><affiliation wicri:level="1"><nlm:aff id="af18">Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster, Germany</nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster</wicri:regionArea><wicri:noRegion>Muenster</wicri:noRegion><wicri:noRegion>Muenster</wicri:noRegion><wicri:noRegion>Muenster</wicri:noRegion></affiliation></author><author><name sortKey="Schwerk, Nicolaus" sort="Schwerk, Nicolaus" uniqKey="Schwerk N" first="Nicolaus" last="Schwerk">Nicolaus Schwerk</name><affiliation wicri:level="3"><nlm:aff id="af19">Clinic for Paediatric Pulmonology, Allergiology and Neonatology, Hannover Medical School, Hannover, Germany</nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Clinic for Paediatric Pulmonology, Allergiology and Neonatology, Hannover Medical School, Hannover</wicri:regionArea><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Hanovre</settlement></placeName></affiliation></author><author><name sortKey="Scigliano, Sergio" sort="Scigliano, Sergio" uniqKey="Scigliano S" first="Sergio" last="Scigliano">Sergio Scigliano</name><affiliation wicri:level="1"><nlm:aff id="af20">Centro Respiratorio, Hospital de Niños Ricardo Gutierrez, Buenos Aires, Argentina</nlm:aff><country xml:lang="fr">Argentine</country><wicri:regionArea>Centro Respiratorio, Hospital de Niños Ricardo Gutierrez, Buenos Aires</wicri:regionArea><wicri:noRegion>Buenos Aires</wicri:noRegion></affiliation></author><author><name sortKey="Werner, Claudius" sort="Werner, Claudius" uniqKey="Werner C" first="Claudius" last="Werner">Claudius Werner</name><affiliation wicri:level="1"><nlm:aff id="af18">Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster, Germany</nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster</wicri:regionArea><wicri:noRegion>Muenster</wicri:noRegion><wicri:noRegion>Muenster</wicri:noRegion><wicri:noRegion>Muenster</wicri:noRegion></affiliation></author><author><name sortKey="Yiallouros, Panayiotis" sort="Yiallouros, Panayiotis" uniqKey="Yiallouros P" first="Panayiotis" last="Yiallouros">Panayiotis Yiallouros</name><affiliation wicri:level="1"><nlm:aff id="af21">Medical School, University of Cyprus, Nicosia, Cyprus</nlm:aff><country xml:lang="fr">Chypre (pays)</country><wicri:regionArea>Medical School, University of Cyprus, Nicosia</wicri:regionArea><wicri:noRegion>Nicosia</wicri:noRegion></affiliation></author><author><name sortKey="Zivkovic, Zorica" sort="Zivkovic, Zorica" uniqKey="Zivkovic Z" first="Zorica" last="Zivkovic">Zorica Zivkovic</name><affiliation wicri:level="1"><nlm:aff id="af22">Children's Hospital for Lung Diseases and TB, Medical Centre “Dr Dragisa Misovic”, Belgrade, Serbia</nlm:aff><country xml:lang="fr">Serbie</country><wicri:regionArea>Children's Hospital for Lung Diseases and TB, Medical Centre “Dr Dragisa Misovic”, Belgrade</wicri:regionArea><wicri:noRegion>Belgrade</wicri:noRegion></affiliation><affiliation wicri:level="1"><nlm:aff id="af23">Faculty of Pharmacy, University Business Academy, Novi Sad, Serbia</nlm:aff><country xml:lang="fr">Serbie</country><wicri:regionArea>Faculty of Pharmacy, University Business Academy, Novi Sad</wicri:regionArea><wicri:noRegion>Novi Sad</wicri:noRegion></affiliation></author><author><name sortKey="Lucas, Jane S" sort="Lucas, Jane S" uniqKey="Lucas J" first="Jane S." last="Lucas">Jane S. Lucas</name><affiliation wicri:level="1"><nlm:aff id="af5">Primary Ciliary Dyskinesia Centre, NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK</nlm:aff><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Primary Ciliary Dyskinesia Centre, NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton</wicri:regionArea><wicri:noRegion>Southampton</wicri:noRegion></affiliation></author><author><name sortKey="Kuehni, Claudia E" sort="Kuehni, Claudia E" uniqKey="Kuehni C" first="Claudia E." last="Kuehni">Claudia E. 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Halbeisen</name><affiliation wicri:level="4"><nlm:aff id="af1">Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland</nlm:aff><country xml:lang="fr">Suisse</country><wicri:regionArea>Institute of Social and Preventive Medicine, University of Bern, Bern</wicri:regionArea><placeName><settlement type="city">Berne</settlement><region type="région" nuts="3">Canton de Berne</region><settlement type="city">Berne</settlement></placeName><orgName type="university">Université de Berne</orgName></affiliation></author><author><name sortKey="Amirav, Israel" sort="Amirav, Israel" uniqKey="Amirav I" first="Israel" last="Amirav">Israel Amirav</name><affiliation wicri:level="1"><nlm:aff id="af3">Dept of Pediatrics, Faculty of Medicine, Bar IIan University, Ramat Gan, Israel</nlm:aff><country xml:lang="fr">Israël</country><wicri:regionArea>Dept of Pediatrics, Faculty of Medicine, Bar IIan University, Ramat Gan</wicri:regionArea><wicri:noRegion>Ramat Gan</wicri:noRegion></affiliation></author><author><name sortKey="Barbato, Angelo" sort="Barbato, Angelo" uniqKey="Barbato A" first="Angelo" last="Barbato">Angelo Barbato</name><affiliation wicri:level="1"><nlm:aff id="af4">Primary Ciliary Dyskinesia Centre, Dept of Women's and Children's Health (SDB), University of Padova, Padova, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Primary Ciliary Dyskinesia Centre, Dept of Women's and Children's Health (SDB), University of Padova, Padova</wicri:regionArea><wicri:noRegion>Padova</wicri:noRegion></affiliation></author><author><name sortKey="Behan, Laura" sort="Behan, Laura" uniqKey="Behan L" first="Laura" last="Behan">Laura Behan</name><affiliation wicri:level="1"><nlm:aff id="af5">Primary Ciliary Dyskinesia Centre, NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK</nlm:aff><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Primary Ciliary Dyskinesia Centre, NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton</wicri:regionArea><wicri:noRegion>Southampton</wicri:noRegion></affiliation></author><author><name sortKey="Boon, Mieke" sort="Boon, Mieke" uniqKey="Boon M" first="Mieke" last="Boon">Mieke Boon</name><affiliation wicri:level="1"><nlm:aff id="af6">Dept of Paediatrics, University Hospital Gasthuisberg, Leuven, Belgium</nlm:aff><country xml:lang="fr">Belgique</country><wicri:regionArea>Dept of Paediatrics, University Hospital Gasthuisberg, Leuven</wicri:regionArea><wicri:noRegion>Leuven</wicri:noRegion></affiliation></author><author><name sortKey="Casaulta, Carmen" sort="Casaulta, Carmen" uniqKey="Casaulta C" first="Carmen" last="Casaulta">Carmen Casaulta</name><affiliation wicri:level="3"><nlm:aff id="af2">Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Bern, Switzerland</nlm:aff><country xml:lang="fr">Suisse</country><wicri:regionArea>Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Bern</wicri:regionArea><placeName><settlement type="city">Berne</settlement><region type="région" nuts="3">Canton de Berne</region></placeName></affiliation></author><author><name sortKey="Clement, Annick" sort="Clement, Annick" uniqKey="Clement A" first="Annick" last="Clement">Annick Clement</name><affiliation wicri:level="3"><nlm:aff id="af7">Paediatric Pulmonary Dept, Trousseau Hospital APHP, Sorbonne Universities and Pierre et Marie Curie University, Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Paediatric Pulmonary Dept, Trousseau Hospital APHP, Sorbonne Universities and Pierre et Marie Curie University, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Crowley, Suzanne" sort="Crowley, Suzanne" uniqKey="Crowley S" first="Suzanne" last="Crowley">Suzanne Crowley</name><affiliation wicri:level="3"><nlm:aff id="af8">Unit for Paediatric Heart, Lung and Allergic Diseases, Rikshospitalet, Oslo, Norway</nlm:aff><country xml:lang="fr">Norvège</country><wicri:regionArea>Unit for Paediatric Heart, Lung and Allergic Diseases, Rikshospitalet, Oslo</wicri:regionArea><placeName><settlement type="city">Oslo</settlement><region nuts="2">Østlandet</region></placeName></affiliation></author><author><name sortKey="Haarman, Eric" sort="Haarman, Eric" uniqKey="Haarman E" first="Eric" last="Haarman">Eric Haarman</name><affiliation wicri:level="3"><nlm:aff id="af9">Dept of Pediatric Pulmonology, VU University Medical Center, Amsterdam, The Netherlands</nlm:aff><country xml:lang="fr">Pays-Bas</country><wicri:regionArea>Dept of Pediatric Pulmonology, VU University Medical Center, Amsterdam</wicri:regionArea><placeName><settlement type="city">Amsterdam</settlement><region nuts="2" type="province">Hollande-Septentrionale</region></placeName></affiliation></author><author><name sortKey="Hogg, Claire" sort="Hogg, Claire" uniqKey="Hogg C" first="Claire" last="Hogg">Claire Hogg</name><affiliation wicri:level="3"><nlm:aff id="af10">Dept of Paediatrics, Primary Ciliary Dyskinesia Centre, Royal Brompton and Harefield Foundation Trust, London, UK</nlm:aff><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Dept of Paediatrics, Primary Ciliary Dyskinesia Centre, Royal Brompton and Harefield Foundation Trust, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Karadag, Bulent" sort="Karadag, Bulent" uniqKey="Karadag B" first="Bulent" last="Karadag">Bulent Karadag</name><affiliation wicri:level="1"><nlm:aff id="af11">Dept of Paediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey</nlm:aff><country xml:lang="fr">Turquie</country><wicri:regionArea>Dept of Paediatric Pulmonology, Marmara University, School of Medicine, Istanbul</wicri:regionArea><wicri:noRegion>Istanbul</wicri:noRegion></affiliation></author><author><name sortKey="Koerner Rettberg, Cordula" sort="Koerner Rettberg, Cordula" uniqKey="Koerner Rettberg C" first="Cordula" last="Koerner-Rettberg">Cordula Koerner-Rettberg</name><affiliation wicri:level="1"><nlm:aff id="af12">Dept of Paediatric Pneumology, University Children's Hospital of Ruhr University Bochum, Bochum, Germany</nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Dept of Paediatric Pneumology, University Children's Hospital of Ruhr University Bochum, Bochum</wicri:regionArea><wicri:noRegion>Bochum</wicri:noRegion><wicri:noRegion>Bochum</wicri:noRegion><wicri:noRegion>Bochum</wicri:noRegion></affiliation></author><author><name sortKey="Leigh, Margaret W" sort="Leigh, Margaret W" uniqKey="Leigh M" first="Margaret W." last="Leigh">Margaret W. Leigh</name><affiliation wicri:level="2"><nlm:aff id="af13">Dept of Pediatrics, UNC School of Medicine, Chapel Hill, NC, USA</nlm:aff><country xml:lang="fr">États-Unis</country><wicri:regionArea>Dept of Pediatrics, UNC School of Medicine, Chapel Hill, NC</wicri:regionArea><placeName><region type="state">Caroline du Nord</region></placeName></affiliation></author><author><name sortKey="Loebinger, Michael R" sort="Loebinger, Michael R" uniqKey="Loebinger M" first="Michael R." last="Loebinger">Michael R. Loebinger</name><affiliation wicri:level="3"><nlm:aff id="af14">Host Defence Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK</nlm:aff><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Host Defence Unit, Royal Brompton and Harefield NHS Foundation Trust, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Mazurek, Henryk" sort="Mazurek, Henryk" uniqKey="Mazurek H" first="Henryk" last="Mazurek">Henryk Mazurek</name><affiliation wicri:level="1"><nlm:aff id="af15">Dept of Pneumonology and Cystic Fibrosis, Institute of Tuberculosis and Lung Disorders, Rabka-Zdrój, Poland</nlm:aff><country xml:lang="fr">Pologne</country><wicri:regionArea>Dept of Pneumonology and Cystic Fibrosis, Institute of Tuberculosis and Lung Disorders, Rabka-Zdrój</wicri:regionArea><wicri:noRegion>Rabka-Zdrój</wicri:noRegion></affiliation></author><author><name sortKey="Morgan, Lucy" sort="Morgan, Lucy" uniqKey="Morgan L" first="Lucy" last="Morgan">Lucy Morgan</name><affiliation wicri:level="4"><nlm:aff id="af16">Dept of Respiratory Medicine, Concord Hospital Clinical School, University of Sydney, Sydney, Australia</nlm:aff><country xml:lang="fr">Australie</country><wicri:regionArea>Dept of Respiratory Medicine, Concord Hospital Clinical School, University of Sydney, Sydney</wicri:regionArea><placeName><settlement type="city">Sydney</settlement><region type="état">Nouvelle-Galles du Sud</region><settlement type="city">Sydney</settlement></placeName><orgName type="university">Université de Sydney</orgName></affiliation></author><author><name sortKey="Nielsen, Kim G" sort="Nielsen, Kim G" uniqKey="Nielsen K" first="Kim G." last="Nielsen">Kim G. Nielsen</name><affiliation wicri:level="3"><nlm:aff id="af17">Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark</nlm:aff><country xml:lang="fr">Danemark</country><wicri:regionArea>Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen</wicri:regionArea><placeName><settlement type="city">Copenhague</settlement><region type="région" nuts="2">Hovedstaden</region></placeName></affiliation></author><author><name sortKey="Omran, Heymut" sort="Omran, Heymut" uniqKey="Omran H" first="Heymut" last="Omran">Heymut Omran</name><affiliation wicri:level="1"><nlm:aff id="af18">Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster, Germany</nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster</wicri:regionArea><wicri:noRegion>Muenster</wicri:noRegion><wicri:noRegion>Muenster</wicri:noRegion><wicri:noRegion>Muenster</wicri:noRegion></affiliation></author><author><name sortKey="Schwerk, Nicolaus" sort="Schwerk, Nicolaus" uniqKey="Schwerk N" first="Nicolaus" last="Schwerk">Nicolaus Schwerk</name><affiliation wicri:level="3"><nlm:aff id="af19">Clinic for Paediatric Pulmonology, Allergiology and Neonatology, Hannover Medical School, Hannover, Germany</nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Clinic for Paediatric Pulmonology, Allergiology and Neonatology, Hannover Medical School, Hannover</wicri:regionArea><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Hanovre</settlement></placeName></affiliation></author><author><name sortKey="Scigliano, Sergio" sort="Scigliano, Sergio" uniqKey="Scigliano S" first="Sergio" last="Scigliano">Sergio Scigliano</name><affiliation wicri:level="1"><nlm:aff id="af20">Centro Respiratorio, Hospital de Niños Ricardo Gutierrez, Buenos Aires, Argentina</nlm:aff><country xml:lang="fr">Argentine</country><wicri:regionArea>Centro Respiratorio, Hospital de Niños Ricardo Gutierrez, Buenos Aires</wicri:regionArea><wicri:noRegion>Buenos Aires</wicri:noRegion></affiliation></author><author><name sortKey="Werner, Claudius" sort="Werner, Claudius" uniqKey="Werner C" first="Claudius" last="Werner">Claudius Werner</name><affiliation wicri:level="1"><nlm:aff id="af18">Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster, Germany</nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster</wicri:regionArea><wicri:noRegion>Muenster</wicri:noRegion><wicri:noRegion>Muenster</wicri:noRegion><wicri:noRegion>Muenster</wicri:noRegion></affiliation></author><author><name sortKey="Yiallouros, Panayiotis" sort="Yiallouros, Panayiotis" uniqKey="Yiallouros P" first="Panayiotis" last="Yiallouros">Panayiotis Yiallouros</name><affiliation wicri:level="1"><nlm:aff id="af21">Medical School, University of Cyprus, Nicosia, Cyprus</nlm:aff><country xml:lang="fr">Chypre (pays)</country><wicri:regionArea>Medical School, University of Cyprus, Nicosia</wicri:regionArea><wicri:noRegion>Nicosia</wicri:noRegion></affiliation></author><author><name sortKey="Zivkovic, Zorica" sort="Zivkovic, Zorica" uniqKey="Zivkovic Z" first="Zorica" last="Zivkovic">Zorica Zivkovic</name><affiliation wicri:level="1"><nlm:aff id="af22">Children's Hospital for Lung Diseases and TB, Medical Centre “Dr Dragisa Misovic”, Belgrade, Serbia</nlm:aff><country xml:lang="fr">Serbie</country><wicri:regionArea>Children's Hospital for Lung Diseases and TB, Medical Centre “Dr Dragisa Misovic”, Belgrade</wicri:regionArea><wicri:noRegion>Belgrade</wicri:noRegion></affiliation><affiliation wicri:level="1"><nlm:aff id="af23">Faculty of Pharmacy, University Business Academy, Novi Sad, Serbia</nlm:aff><country xml:lang="fr">Serbie</country><wicri:regionArea>Faculty of Pharmacy, University Business Academy, Novi Sad</wicri:regionArea><wicri:noRegion>Novi Sad</wicri:noRegion></affiliation></author><author><name sortKey="Lucas, Jane S" sort="Lucas, Jane S" uniqKey="Lucas J" first="Jane S." last="Lucas">Jane S. Lucas</name><affiliation wicri:level="1"><nlm:aff id="af5">Primary Ciliary Dyskinesia Centre, NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK</nlm:aff><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Primary Ciliary Dyskinesia Centre, NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton</wicri:regionArea><wicri:noRegion>Southampton</wicri:noRegion></affiliation></author><author><name sortKey="Kuehni, Claudia E" sort="Kuehni, Claudia E" uniqKey="Kuehni C" first="Claudia E." last="Kuehni">Claudia E. Kuehni</name><affiliation wicri:level="4"><nlm:aff id="af1">Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland</nlm:aff><country xml:lang="fr">Suisse</country><wicri:regionArea>Institute of Social and Preventive Medicine, University of Bern, Bern</wicri:regionArea><placeName><settlement type="city">Berne</settlement><region type="région" nuts="3">Canton de Berne</region><settlement type="city">Berne</settlement></placeName><orgName type="university">Université de Berne</orgName></affiliation></author></analytic><series><title level="j">The European Respiratory Journal</title><idno type="ISSN">0903-1936</idno><idno type="eISSN">1399-3003</idno><imprint><date when="2017">2017</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Data on primary ciliary dyskinesia (PCD) epidemiology is scarce and published studies are characterised by low numbers. In the framework of the European Union project BESTCILIA we aimed to combine all available datasets in a retrospective international PCD cohort (iPCD Cohort).</p><p>We identified eligible datasets by performing a systematic review of published studies containing clinical information on PCD, and by contacting members of past and current European Respiratory Society Task Forces on PCD. We compared the contents of the datasets, clarified definitions and pooled them in a standardised format.</p><p>As of April 2016 the iPCD Cohort includes data on 3013 patients from 18 countries. It includes data on diagnostic evaluations, symptoms, lung function, growth and treatments. Longitudinal data are currently available for 542 patients. The extent of clinical details per patient varies between centres. More than 50% of patients have a definite PCD diagnosis based on recent guidelines. Children aged 10–19 years are the largest age group, followed by younger children (≤9 years) and young adults (20–29 years).</p><p>This is the largest observational PCD dataset available to date. 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Strippoli</name></author><author><name sortKey="Frischer, T" uniqKey="Frischer T">T Frischer</name></author><author><name sortKey="Barbato, A" uniqKey="Barbato A">A Barbato</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Werner, C" uniqKey="Werner C">C Werner</name></author><author><name sortKey="Lablans, M" uniqKey="Lablans M">M Lablans</name></author><author><name sortKey="Ataian, M" uniqKey="Ataian M">M Ataian</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Lucas, Js" uniqKey="Lucas J">JS Lucas</name></author><author><name sortKey="Paff, T" uniqKey="Paff T">T Paff</name></author><author><name sortKey="Goggin, P" uniqKey="Goggin P">P Goggin</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Kartagener, M" uniqKey="Kartagener M">M Kartagener</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Lindberg, S" uniqKey="Lindberg S">S Lindberg</name></author><author><name sortKey="Cervin, A" uniqKey="Cervin A">A Cervin</name></author><author><name sortKey="Runer, T" uniqKey="Runer T">T Runer</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Harris, Pa" uniqKey="Harris P">PA Harris</name></author><author><name sortKey="Taylor, R" uniqKey="Taylor R">R Taylor</name></author><author><name sortKey="Thielke, R" uniqKey="Thielke R">R Thielke</name></author></analytic></biblStruct></listBibl></div1></back></TEI><affiliations><list><country><li>Allemagne</li><li>Argentine</li><li>Australie</li><li>Belgique</li><li>Chypre (pays)</li><li>Danemark</li><li>France</li><li>Israël</li><li>Italie</li><li>Norvège</li><li>Pays-Bas</li><li>Pologne</li><li>Royaume-Uni</li><li>Serbie</li><li>Suisse</li><li>Turquie</li><li>États-Unis</li></country><region><li>Angleterre</li><li>Basse-Saxe</li><li>Canton de Berne</li><li>Caroline du Nord</li><li>Grand Londres</li><li>Hollande-Septentrionale</li><li>Hovedstaden</li><li>Nouvelle-Galles du Sud</li><li>Île-de-France</li><li>Østlandet</li></region><settlement><li>Amsterdam</li><li>Berne</li><li>Copenhague</li><li>Hanovre</li><li>Londres</li><li>Oslo</li><li>Paris</li><li>Sydney</li></settlement><orgName><li>Université de Berne</li><li>Université de Sydney</li></orgName></list><tree><country name="Suisse"><region name="Canton de Berne"><name sortKey="Goutaki, Myrofora" sort="Goutaki, Myrofora" uniqKey="Goutaki M" first="Myrofora" last="Goutaki">Myrofora Goutaki</name></region><name sortKey="Casaulta, Carmen" sort="Casaulta, Carmen" uniqKey="Casaulta C" first="Carmen" last="Casaulta">Carmen Casaulta</name><name sortKey="Goutaki, Myrofora" sort="Goutaki, Myrofora" uniqKey="Goutaki M" first="Myrofora" last="Goutaki">Myrofora Goutaki</name><name sortKey="Halbeisen, Florian S" sort="Halbeisen, Florian S" uniqKey="Halbeisen F" first="Florian S." last="Halbeisen">Florian S. Halbeisen</name><name sortKey="Kuehni, Claudia E" sort="Kuehni, Claudia E" uniqKey="Kuehni C" first="Claudia E." last="Kuehni">Claudia E. Kuehni</name><name sortKey="Maurer, Elisabeth" sort="Maurer, Elisabeth" uniqKey="Maurer E" first="Elisabeth" last="Maurer">Elisabeth Maurer</name></country><country name="Israël"><noRegion><name sortKey="Amirav, Israel" sort="Amirav, Israel" uniqKey="Amirav I" first="Israel" last="Amirav">Israel Amirav</name></noRegion></country><country name="Italie"><noRegion><name sortKey="Barbato, Angelo" sort="Barbato, Angelo" uniqKey="Barbato A" first="Angelo" last="Barbato">Angelo Barbato</name></noRegion></country><country name="Royaume-Uni"><noRegion><name sortKey="Behan, Laura" sort="Behan, Laura" uniqKey="Behan L" first="Laura" last="Behan">Laura Behan</name></noRegion><name sortKey="Hogg, Claire" sort="Hogg, Claire" uniqKey="Hogg C" first="Claire" last="Hogg">Claire Hogg</name><name sortKey="Loebinger, Michael R" sort="Loebinger, Michael R" uniqKey="Loebinger M" first="Michael R." last="Loebinger">Michael R. Loebinger</name><name sortKey="Lucas, Jane S" sort="Lucas, Jane S" uniqKey="Lucas J" first="Jane S." last="Lucas">Jane S. Lucas</name></country><country name="Belgique"><noRegion><name sortKey="Boon, Mieke" sort="Boon, Mieke" uniqKey="Boon M" first="Mieke" last="Boon">Mieke Boon</name></noRegion></country><country name="France"><region name="Île-de-France"><name sortKey="Clement, Annick" sort="Clement, Annick" uniqKey="Clement A" first="Annick" last="Clement">Annick Clement</name></region></country><country name="Norvège"><region name="Østlandet"><name sortKey="Crowley, Suzanne" sort="Crowley, Suzanne" uniqKey="Crowley S" first="Suzanne" last="Crowley">Suzanne Crowley</name></region></country><country name="Pays-Bas"><region name="Hollande-Septentrionale"><name sortKey="Haarman, Eric" sort="Haarman, Eric" uniqKey="Haarman E" first="Eric" last="Haarman">Eric Haarman</name></region></country><country name="Turquie"><noRegion><name sortKey="Karadag, Bulent" sort="Karadag, Bulent" uniqKey="Karadag B" first="Bulent" last="Karadag">Bulent Karadag</name></noRegion></country><country name="Allemagne"><noRegion><name sortKey="Koerner Rettberg, Cordula" sort="Koerner Rettberg, Cordula" uniqKey="Koerner Rettberg C" first="Cordula" last="Koerner-Rettberg">Cordula Koerner-Rettberg</name></noRegion><name sortKey="Omran, Heymut" sort="Omran, Heymut" uniqKey="Omran H" first="Heymut" last="Omran">Heymut Omran</name><name sortKey="Schwerk, Nicolaus" sort="Schwerk, Nicolaus" uniqKey="Schwerk N" first="Nicolaus" last="Schwerk">Nicolaus Schwerk</name><name sortKey="Werner, Claudius" sort="Werner, Claudius" uniqKey="Werner C" first="Claudius" last="Werner">Claudius Werner</name></country><country name="États-Unis"><region name="Caroline du Nord"><name sortKey="Leigh, Margaret W" sort="Leigh, Margaret W" uniqKey="Leigh M" first="Margaret W." last="Leigh">Margaret W. Leigh</name></region></country><country name="Pologne"><noRegion><name sortKey="Mazurek, Henryk" sort="Mazurek, Henryk" uniqKey="Mazurek H" first="Henryk" last="Mazurek">Henryk Mazurek</name></noRegion></country><country name="Australie"><region name="Nouvelle-Galles du Sud"><name sortKey="Morgan, Lucy" sort="Morgan, Lucy" uniqKey="Morgan L" first="Lucy" last="Morgan">Lucy Morgan</name></region></country><country name="Danemark"><region name="Hovedstaden"><name sortKey="Nielsen, Kim G" sort="Nielsen, Kim G" uniqKey="Nielsen K" first="Kim G." last="Nielsen">Kim G. Nielsen</name></region></country><country name="Argentine"><noRegion><name sortKey="Scigliano, Sergio" sort="Scigliano, Sergio" uniqKey="Scigliano S" first="Sergio" last="Scigliano">Sergio Scigliano</name></noRegion></country><country name="Chypre (pays)"><noRegion><name sortKey="Yiallouros, Panayiotis" sort="Yiallouros, Panayiotis" uniqKey="Yiallouros P" first="Panayiotis" last="Yiallouros">Panayiotis Yiallouros</name></noRegion></country><country name="Serbie"><noRegion><name sortKey="Zivkovic, Zorica" sort="Zivkovic, Zorica" uniqKey="Zivkovic Z" first="Zorica" last="Zivkovic">Zorica Zivkovic</name></noRegion><name sortKey="Zivkovic, Zorica" sort="Zivkovic, Zorica" uniqKey="Zivkovic Z" first="Zorica" last="Zivkovic">Zorica Zivkovic</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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