Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children

Barbato, A; Frischer, T; Kuehni, C E; Snijders, D; Azevedo, I; Baktai, G; Bartoloni, L; Eber, E; Escribano, A; Haarman, E; Hesselmar, B; Hogg, C; Jorissen, M; Lucas, J; Nielsen, K G; O'Callaghan, C; Omran, H; Pohunek, P; Strippoli, M-P F and Bush, A (2009). Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children. European respiratory journal, 34(6), pp. 1264-76. Sheffield, UK: European Respiratory Society 10.1183/09031936.00176608

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Primary ciliary dyskinesia (PCD) is associated with abnormal ciliary structure and function, which results in retention of mucus and bacteria in the respiratory tract, leading to chronic oto-sino-pulmonary disease, situs abnormalities and abnormal sperm motility. The diagnosis of PCD requires the presence of the characteristic clinical phenotype and either specific ultrastructural ciliary defects identified by transmission electron microscopy or evidence of abnormal ciliary function. Although the management of children affected with PCD remains uncertain and evidence is limited, it remains important to follow-up these patients with an adequate and shared care system in order to prevent future lung damage. This European Respiratory Society consensus statement on the management of children with PCD formulates recommendations regarding diagnostic and therapeutic approaches in order to permit a more accurate approach in these patients. Large well-designed randomised controlled trials, with clear description of patients, are required in order to improve these recommendations on diagnostic and treatment approaches in this disease.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Social and Preventive Medicine (ISPM)

UniBE Contributor:

Kühni, Claudia and Strippoli, Marie-Pierre

ISSN:

0903-1936

ISBN:

19948909

Publisher:

European Respiratory Society

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 15:09

Last Modified:

10 Sep 2017 14:25

Publisher DOI:

10.1183/09031936.00176608

PubMed ID:

19948909

Web of Science ID:

000272625000005

BORIS DOI:

10.7892/boris.30355

URI:

https://boris.unibe.ch/id/eprint/30355 (FactScience: 192914)

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