Management of primary ciliary dyskinesia in European children: recommendations and clinical practice

Strippoli, Marie-Pierre F; Frischer, Thomas; Barbato, Angelo; Snijders, Deborah; Maurer, Elisabeth; Lucas, Jane S A; Eber, Ernst; Karadag, Bulent; Pohunek, Petr; Zivkovic, Zorica; Escribano, Amparo; O'Callaghan, Chris; Bush, Andrew; Kuehni, Claudia E; ERS Task Force onPrimary Ciliary Dyskinesia in Children, (2012). Management of primary ciliary dyskinesia in European children: recommendations and clinical practice. European respiratory journal, 39(6), pp. 1482-91. Sheffield, UK: European Respiratory Society 10.1183/09031936.00073911

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The European Respiratory Society Task Force on primary ciliary dyskinesia (PCD) in children recently published recommendations for diagnosis and management. This paper compares these recommendations with current clinical practice in Europe. Questionnaires were returned by 194 paediatric respiratory centres caring for PCD patients in 26 countries. In most countries, PCD care was not centralised, with a median (interquartile range) of 4 (2-9) patients treated per centre. Overall, 90% of centres had access to nasal or bronchial mucosal biopsy. Samples were analysed by electron microscopy (77%) and ciliary function tests (57%). Nasal nitric oxide was used for screening in 46% of centres and saccharine tests in 36%. Treatment approaches varied widely, both within and between countries. European region, size of centre and the country's general government expenditure on health partly defined availability of advanced diagnostic tests and choice of treatments. In conclusion, we found substantial heterogeneity in management of PCD within and between countries, and poor concordance with current recommendations. This demonstrates how essential it is to standardise management and decrease inequality between countries. Our results also demonstrate the urgent need for research: to simplify PCD diagnosis, to understand the natural history and to test the effectiveness of interventions.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Social and Preventive Medicine

UniBE Contributor:

Strippoli, Marie-Pierre; Maurer, Elisabeth and Kühni, Claudia

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0903-1936

Publisher:

European Respiratory Society

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:35

Last Modified:

26 Jan 2015 04:21

Publisher DOI:

10.1183/09031936.00073911

PubMed ID:

22282549

Web of Science ID:

000305369900030

BORIS DOI:

10.7892/boris.13996

URI:

https://boris.unibe.ch/id/eprint/13996 (FactScience: 220718)

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