PT  - JOURNAL ARTICLE
AU  - Marie-Pierre F. Strippoli
AU  - Thomas Frischer
AU  - Angelo Barbato
AU  - Deborah Snijders
AU  - Elisabeth Maurer
AU  - Jane S.A. Lucas
AU  - Ernst Eber
AU  - Bulent Karadag
AU  - Petr Pohunek
AU  - Zorica Zivkovic
AU  - Amparo Escribano
AU  - Chris O’Callaghan
AU  - Andrew Bush
AU  - Claudia E. Kuehni
TI  - Management of primary ciliary dyskinesia in European children: recommendations and clinical practice
AID  - 10.1183/09031936.00073911
DP  - 2012 Jun 01
TA  - European Respiratory Journal
PG  - 1482--1491
VI  - 39
IP  - 6
4099  - http://erj.ersjournals.com/content/39/6/1482.short
4100  - http://erj.ersjournals.com/content/39/6/1482.full
SO  - Eur Respir J2012 Jun 01; 39
AB  - 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.