RT Journal Article SR Electronic T1 The international primary ciliary dyskinesia cohort (iPCD Cohort): methods and first results JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 1601181 DO 10.1183/13993003.01181-2016 VO 49 IS 1 A1 Goutaki, Myrofora A1 Maurer, Elisabeth A1 Halbeisen, Florian S. A1 Amirav, Israel A1 Barbato, Angelo A1 Behan, Laura A1 Boon, Mieke A1 Casaulta, Carmen A1 Clement, Annick A1 Crowley, Suzanne A1 Haarman, Eric A1 Hogg, Claire A1 Karadag, Bulent A1 Koerner-Rettberg, Cordula A1 Leigh, Margaret W. A1 Loebinger, Michael R. A1 Mazurek, Henryk A1 Morgan, Lucy A1 Nielsen, Kim G. A1 Omran, Heymut A1 Schwerk, Nicolaus A1 Scigliano, Sergio A1 Werner, Claudius A1 Yiallouros, Panayiotis A1 Zivkovic, Zorica A1 Lucas, Jane S. A1 Kuehni, Claudia E. YR 2017 UL http://erj.ersjournals.com/content/49/1/1601181.abstract AB 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.The iPCD Cohort offers a unique opportunity to study PCD in an international retrospective cohort of >3000 patients http://ow.ly/rn0m304Jgsu