Management of primary ciliary dyskinesia: current practice and future perspectives
Management of primary ciliary dyskinesia: current practice and future perspectives
Primary ciliary dyskinesia (PCD) is an inherited disease, genetically and clinically heterogeneous, characterised by abnormal motile ciliary function. Although early symptoms predominantly effect the airways and most patients develop bronchiectasis, PCD is a multisystem disease and management
must be multidisciplinary. Particularly relevant are chronic upper and lower respiratory tract symptoms, laterality disorders, cardiac manifestations and fertility. There is no strong evidence for the effectiveness of different therapies for PCD. This article highlights central aspects of PCD management, describes current practice and gives an overview on ongoing efforts to improve the evidence
European Respiratory Society
Kuehni, Claudia E.
ac67c925-ee32-429d-a3b5-c244daa314b4
Goutaki, Myrofora
60fbeefc-dbb1-429c-b81a-3c35d368db64
Rubbo, Bruna
dc31cd48-3d84-41ab-a8b8-351c9914dca4
Lucas, Jane
5cb3546c-87b2-4e59-af48-402076e25313
9 May 2018
Kuehni, Claudia E.
ac67c925-ee32-429d-a3b5-c244daa314b4
Goutaki, Myrofora
60fbeefc-dbb1-429c-b81a-3c35d368db64
Rubbo, Bruna
dc31cd48-3d84-41ab-a8b8-351c9914dca4
Lucas, Jane
5cb3546c-87b2-4e59-af48-402076e25313
Kuehni, Claudia E., Goutaki, Myrofora, Rubbo, Bruna and Lucas, Jane
(2018)
Management of primary ciliary dyskinesia: current practice and future perspectives.
In,
ERS Monograph: Bronchiectasis.
European Respiratory Society.
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Book Section
Abstract
Primary ciliary dyskinesia (PCD) is an inherited disease, genetically and clinically heterogeneous, characterised by abnormal motile ciliary function. Although early symptoms predominantly effect the airways and most patients develop bronchiectasis, PCD is a multisystem disease and management
must be multidisciplinary. Particularly relevant are chronic upper and lower respiratory tract symptoms, laterality disorders, cardiac manifestations and fertility. There is no strong evidence for the effectiveness of different therapies for PCD. This article highlights central aspects of PCD management, describes current practice and gives an overview on ongoing efforts to improve the evidence
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Published date: 9 May 2018
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Local EPrints ID: 421977
URI: http://eprints.soton.ac.uk/id/eprint/421977
PURE UUID: 3bc1e2ca-68c9-4f7f-985a-bb01a7bdc1cc
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Date deposited: 12 Jul 2018 16:30
Last modified: 16 Mar 2024 04:22
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Contributors
Author:
Claudia E. Kuehni
Author:
Myrofora Goutaki
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