title: Management of primary ciliary dyskinesia in European children: recommendations and clinical practice.
abstract: 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.
authors.affiliation: Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
authors.forename: Marie-Pierre F
authors.initials: MP
authors.lastname: Strippoli
authors.forename: Thomas
authors.initials: T
authors.lastname: Frischer
authors.forename: Angelo
authors.initials: A
authors.lastname: Barbato
authors.forename: Deborah
authors.initials: D
authors.lastname: Snijders
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Maurer
authors.forename: Jane S A
authors.initials: JS
authors.lastname: Lucas
authors.forename: Ernst
authors.initials: E
authors.lastname: Eber
authors.forename: Bulent
authors.initials: B
authors.lastname: Karadag
authors.forename: Petr
authors.initials: P
authors.lastname: Pohunek
authors.forename: Zorica
authors.initials: Z
authors.lastname: Zivkovic
authors.forename: Amparo
authors.initials: A
authors.lastname: Escribano
authors.forename: Chris
authors.initials: C
authors.lastname: O'Callaghan
authors.forename: Andrew
authors.initials: A
authors.lastname: Bush
authors.forename: Claudia E
authors.initials: CE
authors.lastname: Kuehni
mesh_terms: D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000628:therapy; D005060:Europe; D005260:Female; D019538:Health Care Surveys; D006801:Humans; D007619:Kartagener Syndrome / Q000175:diagnosis* / Q000628:therapy*; D008297:Male; D009569:Nitric Oxide / Q000032:analysis; D017410:Practice Guidelines as Topic; D020545:Respiratory Mucosa / Q000473:pathology / Q000648:ultrastructure; D012439:Saccharin; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
chemical_list: D009569:Nitric Oxide; D012439:Saccharin