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Title: An international registry for primary ciliary dyskinesia
Authors: Werner, Claudius 
Lablans, Martin 
Ataian, Maximilian 
Raidt, Johanna 
Wallmeier, Julia 
Große-Onnebrink, Jörg 
Kuehni, Claudia 
Haarman, Eric Gerardus 
Leigh, Margaret W. 
Quittner, Alexandra 
Lucas, Jane 
Hogg, Claire L. 
Witt, Michal 
Priftis, Costas 
Yiallouros, Panayiotis K. 
Nielsen, Kim Gjerum 
Santamaria, Francesca 
Ückert, Frank 
Omran, Heymut 
Keywords: Αntibiotic agent;Bronchodilating agent;Corticosteroid
Category: Other Medical Sciences
Field: Medical and Health Sciences
Issue Date: 1-Mar-2016
Publisher: European Respiratory Society
Source: European Respiratory Journal,2016, Volume 47, Issue 3,Pages 849-859
DOI: 10.1183/13993003.00776-2015
Abstract: 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 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.
ISSN: 09031936
Rights: © ERS 2016
Type: Article
Appears in Collections:Άρθρα/Articles

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