RT Journal Article
SR Electronic
T1 Management of primary ciliary dyskinesia in European children: recommendations and clinical practice
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1482
OP 1491
DO 10.1183/09031936.00073911
VO 39
IS 6
A1 Marie-Pierre F. Strippoli
A1 Thomas Frischer
A1 Angelo Barbato
A1 Deborah Snijders
A1 Elisabeth Maurer
A1 Jane S.A. Lucas
A1 Ernst Eber
A1 Bulent Karadag
A1 Petr Pohunek
A1 Zorica Zivkovic
A1 Amparo Escribano
A1 Chris O’Callaghan
A1 Andrew Bush
A1 Claudia E. Kuehni
YR 2012
UL http://erj.ersjournals.com/content/39/6/1482.abstract
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.