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Management of primary ciliary dyskinesia: current practice and future perspectives

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.
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Goutaki, Myrofora
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Rubbo, Bruna
dc31cd48-3d84-41ab-a8b8-351c9914dca4
Lucas, Jane
5cb3546c-87b2-4e59-af48-402076e25313
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.

Record type: 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
ORCID for Bruna Rubbo: ORCID iD orcid.org/0000-0002-1629-8601
ORCID for Jane Lucas: ORCID iD orcid.org/0000-0001-8701-9975

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Date deposited: 12 Jul 2018 16:30
Last modified: 12 Jun 2024 01:48

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Contributors

Author: Claudia E. Kuehni
Author: Myrofora Goutaki
Author: Bruna Rubbo ORCID iD
Author: Jane Lucas ORCID iD

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