Management of primary ciliary dyskinesia in European children: recommendations and clinical practice
Management of primary ciliary dyskinesia in European children: recommendations and clinical practice
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 of 4 (IQR 2-9) patients treated per centre. Overall, 90% of centres had access to nasal or bronchial mucosal biopsy. Samples were analyse by electron microscopy (77%), ciliary function tests (57%) or both (84%). Nasal nitric oxide for screening was used in 46% of centres, 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.
bronchiectasis, ciliary motility disorders, diagnosis, kartagener syndrome, primary ciliary dyskinesia, therapy
1482-1491
Strippoli, Marie-Pierre F.
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Frischer, Thomas
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Barbato, Angelo
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Snijders, Deborah
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Maurer, Elisabeth
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Lucas, Jane S.A.
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Eber, Ernst
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Karadag, Bulent
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Pohunek, Petr
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Zivkovic, Zorica
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Escribano, Amparo
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O'Callaghan, Chris
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Bush, Andrew
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Kuehni, Claudia E.
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Strippoli, Marie-Pierre F.
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Frischer, Thomas
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Barbato, Angelo
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Snijders, Deborah
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Maurer, Elisabeth
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Lucas, Jane S.A.
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Eber, Ernst
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Karadag, Bulent
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Pohunek, Petr
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Zivkovic, Zorica
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Escribano, Amparo
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O'Callaghan, Chris
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Bush, Andrew
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Kuehni, Claudia E.
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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 and Kuehni, Claudia E.
(2012)
Management of primary ciliary dyskinesia in European children: recommendations and clinical practice.
European Respiratory Journal, 39 (6), .
(doi:10.1183/09031936.00073911).
(PMID:22282549)
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 of 4 (IQR 2-9) patients treated per centre. Overall, 90% of centres had access to nasal or bronchial mucosal biopsy. Samples were analyse by electron microscopy (77%), ciliary function tests (57%) or both (84%). Nasal nitric oxide for screening was used in 46% of centres, 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.
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e-pub ahead of print date: 26 January 2012
Additional Information:
Management of PCD: European survey
Keywords:
bronchiectasis, ciliary motility disorders, diagnosis, kartagener syndrome, primary ciliary dyskinesia, therapy
Organisations:
Clinical & Experimental Sciences
Identifiers
Local EPrints ID: 209415
URI: http://eprints.soton.ac.uk/id/eprint/209415
ISSN: 0903-1936
PURE UUID: 330ff825-58c0-4b0d-880e-87fb84677faa
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Date deposited: 30 Jan 2012 12:36
Last modified: 15 Mar 2024 03:12
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Contributors
Author:
Marie-Pierre F. Strippoli
Author:
Thomas Frischer
Author:
Angelo Barbato
Author:
Deborah Snijders
Author:
Elisabeth Maurer
Author:
Ernst Eber
Author:
Bulent Karadag
Author:
Petr Pohunek
Author:
Zorica Zivkovic
Author:
Amparo Escribano
Author:
Chris O'Callaghan
Author:
Andrew Bush
Author:
Claudia E. Kuehni
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