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Factors influencing age at diagnosis of primary ciliary dyskinesia in European children

Kuehni, C. E., Frischer, T., Strippoli, M-P.F., Maurer, E., Bush, Andrew, Nielsen, K.G., Escribano, A., Lucas, J.S.A., Yiallouros, P., Omran, H., Eber, E., O'Callaghan, C., Snijders, D. and Barbato, A. (2010) Factors influencing age at diagnosis of primary ciliary dyskinesia in European children European Respiratory Journal, 36, (6), pp. 1248-1258. (doi:10.1183/09031936.00001010). (PMID:20530032).

Record type: Article

Abstract

Primary ciliary dyskinesia (PCD) is a hereditary disorder of mucociliary clearance causing chronic upper and lower airways disease. We determined the number of patients with diagnosed PCD across Europe, described age at diagnosis and determined risk factors for late diagnosis. Centres treating children with PCD in Europe answered questionnaires and provided anonymous patient lists. In total, 223 centres from 26 countries reported 1,009 patients aged < 20 yrs. Reported cases per million children (for 5-14 yr olds) were highest in Cyprus (111), Switzerland (47) and Denmark (46). Overall, 57% were males and 48% had situs inversus. Median age at diagnosis was 5.3 yrs, lower in children with situs inversus (3.5 versus 5.8 yrs; p < 0.001) and in children treated in large centres (4.1 versus 4.8 yrs; p = 0.002). Adjusted age at diagnosis was 5.0 yrs in Western Europe, 4.8 yrs in the British Isles, 5.5 yrs in Northern Europe, 6.8 yrs in Eastern Europe and 6.5 yrs in Southern Europe (p < 0.001). This strongly correlated with general government expenditures on health (p < 0.001). This European survey suggests that PCD in children is under-diagnosed and diagnosed late, particularly in countries with low health expenditures. Prospective studies should assess the impact this delay might have on patient prognosis and on health economic costs across Europe.

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Published date: December 2010
Keywords: bronchiectasis, ciliary motility disorders, diagnosis, epidemiology, kartagener syndrome, primary ciliary dyskinesia
Organisations: Infection Inflammation & Immunity

Identifiers

Local EPrints ID: 173537
URI: http://eprints.soton.ac.uk/id/eprint/173537
ISSN: 0903-1936
PURE UUID: c04c57fe-47b8-4b02-98a7-8209faf9c2fb

Catalogue record

Date deposited: 04 Feb 2011 11:55
Last modified: 27 Sep 2017 16:32

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Contributors

Author: C. E. Kuehni
Author: T. Frischer
Author: M-P.F. Strippoli
Author: E. Maurer
Author: Andrew Bush
Author: K.G. Nielsen
Author: A. Escribano
Author: J.S.A. Lucas
Author: P. Yiallouros
Author: H. Omran
Author: E. Eber
Author: C. O'Callaghan
Author: D. Snijders
Author: A. Barbato

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