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An international registry for primary ciliary dyskinesia

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  1. SPEF2- And HYDIN-mutant cilia lack the central pair-associated protein SPEF2, aiding primary ciliary dyskinesia diagnostics

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  2. Primary ciliary dyskinesia patients have the same P. aeruginosa clone in sinuses and lungs

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  3. The Controversies and Difficulties of Diagnosing Primary Ciliary Dyskinesia

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  4. The impact of mannose-binding lectin polymorphisms on lung function in primary ciliary dyskinesia

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  5. Long-Term Lung Function and Exercise Capacity in Postinfectious chILD

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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
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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.

Original languageEnglish
JournalEuropean Respiratory Journal
Volume47
Issue number3
Pages (from-to)849-59
Number of pages11
ISSN0903-1936
DOIs
Publication statusPublished - Mar 2016

    Research areas

  • Adolescent, Adult, Age Distribution, Aged, Child, Child, Preschool, Disease Progression, Europe, Female, Forced Expiratory Volume, Humans, Incidence, Infant, Internet, Intersectoral Collaboration, Kartagener Syndrome, Male, Middle Aged, North America, Patient Selection, Registries, Young Adult, Journal Article, Observational Study, Research Support, Non-U.S. Gov't

ID: 49874218