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An international registry for primary ciliary dyskinesia

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

DOI

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  • Claudius Werner
  • Martin Lablans
  • Maximilian Ataian
  • Johanna Raidt
  • Julia Wallmeier
  • Jörg Große-Onnebrink
  • Claudia E Kuehni
  • Eric G Haarman
  • Margaret W Leigh
  • Alexandra L Quittner
  • Jane S Lucas
  • Claire Hogg
  • Michal Witt
  • Kostas N Priftis
  • Panayiotis Yiallouros
  • Kim G Nielsen
  • Francesca Santamaria
  • Frank Ückert
  • Heymut Omran
Vis graf over relationer

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder leading to chronic upper and lower airway disease. Fundamental data on epidemiology, clinical presentation, course and treatment strategies are lacking in PCD. We have established an international PCD registry to realise an unmet need for an international platform to systematically collect data on incidence, clinical presentation, treatment and disease course.The registry was launched in January 2014. We used internet technology to ensure easy online access using a web browser under www.pcdregistry.eu. Data from 201 patients have been collected so far. The database is comprised of a basic data form including demographic and diagnostic information, and visit forms designed to monitor the disease course.To establish a definite PCD diagnosis, we used strict diagnostic criteria, which required two to three diagnostic methods in addition to classical clinical symptoms. Preliminary analysis of lung function data demonstrated a mean annual decline of percentage predicted forced expiratory volume in 1 s of 0.59% (95% CI 0.98-0.22).Here, we present the development of an international PCD registry as a new promising tool to advance the understanding of this rare disorder, to recruit candidates for research studies and ultimately to improve PCD care.

OriginalsprogEngelsk
TidsskriftEuropean Respiratory Journal
Vol/bind47
Udgave nummer3
Sider (fra-til)849-59
Antal sider11
ISSN0903-1936
DOI
StatusUdgivet - mar. 2016

ID: 49874218