Description of data collected in the international primary ciliary dyskinesia cohort (iPCD Cohort) and centres contributing to the different modules

Type of dataCollected variablesContributing centres#
General informationPatient and centre identifiers, and demographic data (e.g. date of birth, sex, ethnicity, date of last follow-up, date last known alive)All centres
DiagnosticsDiagnostic status of PCD, date of diagnosis, dates, and results of diagnostics test performed (e.g. nNO testing, VM, TEM, genetic testing)All centres
Baseline characteristicsInformation on PCD defects (e.g. situs anomalies, congenital heart defects, brain cilia dysfunction, retinitis pigmentosa, renal problems, fertility problems) and relevant comorbidities unconnected with PCDAll centres
Lung functionLung function date, somatometric measurement, spirometric measurements and other lung function tests (e.g. multiple breath washout)All centres except those in Northern America
Clinical manifestationsDate of clinical visit, information on symptoms and signs (e.g. cough, upper and lower respiratory infections, hearing impairment), exercise limitation and active or passive smoking within the last 3 monthsAR, AU, CH, CY, DE2, DE3, DK, FR, IL, IT, NL, NO, PL, RS, TR, UK2, UK3
TherapyDate of prescription, information on inhaled and nasal medication (e.g. bronchodilators, inhaled corticosteroids, nasal sprays), antibiotics prescription, oxygen therapy, mechanical ventilation and physiotherapyAU, CH, CY, DE3, IL, IT, NO, PL, RS, TR, UK3
MicrobiologyDate of microbiology examination, type of sample and isolated bacteriaAR, BE, CH, CY, DE2, DE3, DK, NO, PL, RS, TR, UK2, UK3
ImagingDate of chest imaging examination, type of chest imaging, information on findings (e.g. bronchiectases, atelectases, infiltrations) and information on other types of imagingBE, CH, CY, DE2, DE3, FR, IL, IT, NO, RS, TR, UK2, UK3
Surgical interventionsDate and type of surgeryAR, AU, BE, CH, CY, DE3, IT, NL, NO, RS, TR
Neonatal periodSymptoms and signs of neonatal period (e.g. neonatal respiratory distress syndrome, neonatal cough, neonatal rhinitis), need for intensive care and ventilationAU, BE, CH, CY, DE2, DE3, FR, IL, IT, NL, NO, RS, TR, UK3
Family historyNumber of siblings, PCD affected siblings and other family members, patient ID of PCD affected family members, and relationship of affected family membersAR, AU, BE, CH, CY, DE2, IL, IT, NO, RS, TR, UK3
  • nNO: nasal nitric oxide; VM: light or high-frequency video microscopy; TEM: transmission electron microscopy. #: AR: Argentina; AU: Australia; BE: Belgium; CH: Switzerland; CY: Cyprus; DE1: Bochum, Germany, DE2: Muenster, Germany; DE3: Hannover, Germany; DK: Denmark; FR: France; IL: Israel; IT: Italy; NL: the Netherlands; NO: Norway; PL: Poland; RS: Serbia; TR: Turkey; UK1: Paediatric Pulmonology Dept, Brompton, UK; UK2: Adult Pulmonology Dept, Brompton, UK; UK3: Southampton, UK. : mandatory data for all centres.