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

Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children
Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children
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
consensus statement, diagnosis, primary ciliary dyskinesia, treatment
0903-1936
1264-1276
Barbato, A.
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Frischer, T.
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Keuhni, C.E.
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Snijders, D.
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Azevedo, I.
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Baktai, G.
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Bartoloni, L.
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Eber, E.
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Escribano, A.
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Haarman, E.
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Hesselmar, B.
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Hogg, C.
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Jorissen, M.
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Lucas, J.
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Nielsen, K.G.
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O'Callaghan, C.
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Omran, H.
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Pohunek, P.
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Strippoli, M-P.F.
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Bush, A.
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Barbato, A.
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Frischer, T.
643dd3e6-8727-44bb-8867-35120e97da5d
Keuhni, C.E.
80b1f7f6-761d-44f6-b171-9f719865623b
Snijders, D.
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Azevedo, I.
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Baktai, G.
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Bartoloni, L.
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Eber, E.
81da5cdf-cdc0-48ee-8d03-5688aa0a6fde
Escribano, A.
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Haarman, E.
953b1ca1-3218-435c-9a0e-32e7b2d95798
Hesselmar, B.
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Hogg, C.
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Jorissen, M.
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Lucas, J.
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Nielsen, K.G.
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O'Callaghan, C.
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Omran, H.
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Pohunek, P.
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Strippoli, M-P.F.
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Bush, A.
010aeb4e-ec67-4a69-bf15-c722a3b6354f

Barbato, A., Frischer, T., Keuhni, 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), 1264-1276. (doi:10.1183/09031936.00176608). (PMID:19948909)

Record type: Article

Abstract

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

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Published date: December 2009
Keywords: consensus statement, diagnosis, primary ciliary dyskinesia, treatment

Identifiers

Local EPrints ID: 72713
URI: https://eprints.soton.ac.uk/id/eprint/72713
ISSN: 0903-1936
PURE UUID: 9c83d567-4b55-4d02-9296-6911287ba8f3

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Date deposited: 23 Feb 2010
Last modified: 11 Nov 2019 21:22

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Contributors

Author: A. Barbato
Author: T. Frischer
Author: C.E. Keuhni
Author: D. Snijders
Author: I. Azevedo
Author: G. Baktai
Author: L. Bartoloni
Author: E. Eber
Author: A. Escribano
Author: E. Haarman
Author: B. Hesselmar
Author: C. Hogg
Author: M. Jorissen
Author: J. Lucas
Author: K.G. Nielsen
Author: C. O'Callaghan
Author: H. Omran
Author: P. Pohunek
Author: M-P.F. Strippoli
Author: A. Bush

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