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Nitric oxide in primary ciliary dyskinesia.

Nitric oxide in primary ciliary dyskinesia.
Nitric oxide in primary ciliary dyskinesia.
Nitric oxide is continually synthesized in the respiratory epithelium and is upregulated in response to infection or inflammation. Primary ciliary dyskinesia is characterized by recurrent sinopulmonary infections due to impaired mucociliary clearance. Despite chronic infections, nasal nitric oxide in such patients is markedly reduced and is used as a screening test for this condition. These low levels were first described over 15 years ago but the underlying mechanisms have yet to be fully elucidated. We review epithelial nitric oxide synthesis, release and measurement in the upper airways with particular reference to primary ciliary dyskinesia. The key hypotheses that have been proposed to explain the low levels in this condition are explored and the potential benefits of augmenting airway nitric oxide levels are considered. Further work in these patients clarifying both whether the respiratory epithelium is able to biosynthesise normal levels of nitric oxide and the role played by abnormalities in the anatomy of the paranasal sinuses is essential. While nitric oxide augmentation is unlikely to be beneficial in common primary ciliary dyskinesia phenotypes, it has potential in the treatment of secondary dyskinesias and may also improve treatment of bacterial infections, particularly where biofilms are implicated.
nasal nitric oxide, nitric oxide augmentation, nitric oxide synthase
0903-1936
1024-1032
Walker, Woolf T.
58aae223-5b0e-4f34-9ee7-58bb68278c3a
Jackson, Claire L.
ca0c242e-3638-4949-a0cb-f41e36067b8f
Lackie, Peter M.
4afbbe1a-22a6-4ceb-8cad-f3696dc43a7a
Hogg, Claire
78881fd2-dbe9-4c28-b050-3387c163df1e
Lucas, Jane S.
5cb3546c-87b2-4e59-af48-402076e25313
Walker, Woolf T.
58aae223-5b0e-4f34-9ee7-58bb68278c3a
Jackson, Claire L.
ca0c242e-3638-4949-a0cb-f41e36067b8f
Lackie, Peter M.
4afbbe1a-22a6-4ceb-8cad-f3696dc43a7a
Hogg, Claire
78881fd2-dbe9-4c28-b050-3387c163df1e
Lucas, Jane S.
5cb3546c-87b2-4e59-af48-402076e25313

Walker, Woolf T., Jackson, Claire L., Lackie, Peter M., Hogg, Claire and Lucas, Jane S. (2012) Nitric oxide in primary ciliary dyskinesia. European Respiratory Journal, 40 (4), 1024-1032. (doi:10.1183/09031936.00176111). (PMID:22408195)

Record type: Article

Abstract

Nitric oxide is continually synthesized in the respiratory epithelium and is upregulated in response to infection or inflammation. Primary ciliary dyskinesia is characterized by recurrent sinopulmonary infections due to impaired mucociliary clearance. Despite chronic infections, nasal nitric oxide in such patients is markedly reduced and is used as a screening test for this condition. These low levels were first described over 15 years ago but the underlying mechanisms have yet to be fully elucidated. We review epithelial nitric oxide synthesis, release and measurement in the upper airways with particular reference to primary ciliary dyskinesia. The key hypotheses that have been proposed to explain the low levels in this condition are explored and the potential benefits of augmenting airway nitric oxide levels are considered. Further work in these patients clarifying both whether the respiratory epithelium is able to biosynthesise normal levels of nitric oxide and the role played by abnormalities in the anatomy of the paranasal sinuses is essential. While nitric oxide augmentation is unlikely to be beneficial in common primary ciliary dyskinesia phenotypes, it has potential in the treatment of secondary dyskinesias and may also improve treatment of bacterial infections, particularly where biofilms are implicated.

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Published date: 9 March 2012
Keywords: nasal nitric oxide, nitric oxide augmentation, nitric oxide synthase
Organisations: Clinical & Experimental Sciences

Identifiers

Local EPrints ID: 336183
URI: http://eprints.soton.ac.uk/id/eprint/336183
ISSN: 0903-1936
PURE UUID: a9206f4f-c439-4925-930f-1ee05e0e1095
ORCID for Peter M. Lackie: ORCID iD orcid.org/0000-0001-7138-3764
ORCID for Jane S. Lucas: ORCID iD orcid.org/0000-0001-8701-9975

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Date deposited: 16 Mar 2012 14:29
Last modified: 15 Mar 2024 03:12

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Contributors

Author: Woolf T. Walker
Author: Claire L. Jackson
Author: Peter M. Lackie ORCID iD
Author: Claire Hogg
Author: Jane S. Lucas ORCID iD

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