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Pulmonary radioaerosol mucociliary clearance in primary ciliary dyskinesia

Pulmonary radioaerosol mucociliary clearance in primary ciliary dyskinesia
Pulmonary radioaerosol mucociliary clearance in primary ciliary dyskinesia
To the Editor:

Primary ciliary dyskinesia (PCD) is a genetic condition affecting one in 10 000–40 000 people from birth [1]; cilia fail to beat, and the airway clearance of mucus and debris is severely impaired. If untreated, this results in progressive lung infection leading to bronchiectasis and ultimately respiratory failure. Additionally, delayed diagnosis has implications for genetic counselling, appropriate management of glue ear and fertility advice. Early diagnosis and appropriate treatment are believed to improve outcome. The diagnosis of PCD is highly specialised and results can remain inconclusive, despite state of the art equipment and diagnostic techniques. A European consensus statement [2, 3] highlighted that there is no “gold-standard” diagnostic test; diagnosis requires expert review of clinical history and screening tests (nasal nitric oxide measurement) alongside analysis of ciliary function and ultrastructure [2]. It is recommended that ciliary activity of respiratory epithelial cells obtained by nasal or bronchial brushing is recorded using a high-speed video camera mounted on a microscope. The images are played back in slow motion to…
0903-1936
533-535
Walker, Woolf T.
58aae223-5b0e-4f34-9ee7-58bb68278c3a
Young, Aneurin
1de8b05a-0468-468c-bf08-b63ecbf63356
Bennett, Michael
6df5585a-3d93-4870-8797-389759fc82c7
Guy, Matthew
a0a4b3bc-2ca1-4ab3-a428-3dd20435e4c8
Carroll, Mary P.
f7e407a5-f7fa-4efd-a7f1-8e6140b86f50
Fleming, John
9dfe6059-3383-4621-9ef4-4ea221640b55
Conway, Joy
bbe9a2e4-fb85-4d4a-a38c-0c1832c32d06
Lucas, Jane S
5cb3546c-87b2-4e59-af48-402076e25313
Walker, Woolf T.
58aae223-5b0e-4f34-9ee7-58bb68278c3a
Young, Aneurin
1de8b05a-0468-468c-bf08-b63ecbf63356
Bennett, Michael
6df5585a-3d93-4870-8797-389759fc82c7
Guy, Matthew
a0a4b3bc-2ca1-4ab3-a428-3dd20435e4c8
Carroll, Mary P.
f7e407a5-f7fa-4efd-a7f1-8e6140b86f50
Fleming, John
9dfe6059-3383-4621-9ef4-4ea221640b55
Conway, Joy
bbe9a2e4-fb85-4d4a-a38c-0c1832c32d06
Lucas, Jane S
5cb3546c-87b2-4e59-af48-402076e25313

Walker, Woolf T., Young, Aneurin, Bennett, Michael, Guy, Matthew, Carroll, Mary P., Fleming, John, Conway, Joy and Lucas, Jane S (2014) Pulmonary radioaerosol mucociliary clearance in primary ciliary dyskinesia. European Respiratory Journal, 44 (2), 533-535. (doi:10.1183/09031936.00011814). (PMID:24791827)

Record type: Article

Abstract

To the Editor:

Primary ciliary dyskinesia (PCD) is a genetic condition affecting one in 10 000–40 000 people from birth [1]; cilia fail to beat, and the airway clearance of mucus and debris is severely impaired. If untreated, this results in progressive lung infection leading to bronchiectasis and ultimately respiratory failure. Additionally, delayed diagnosis has implications for genetic counselling, appropriate management of glue ear and fertility advice. Early diagnosis and appropriate treatment are believed to improve outcome. The diagnosis of PCD is highly specialised and results can remain inconclusive, despite state of the art equipment and diagnostic techniques. A European consensus statement [2, 3] highlighted that there is no “gold-standard” diagnostic test; diagnosis requires expert review of clinical history and screening tests (nasal nitric oxide measurement) alongside analysis of ciliary function and ultrastructure [2]. It is recommended that ciliary activity of respiratory epithelial cells obtained by nasal or bronchial brushing is recorded using a high-speed video camera mounted on a microscope. The images are played back in slow motion to…

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e-pub ahead of print date: 2 May 2014
Published date: 1 August 2014
Organisations: Clinical & Experimental Sciences

Identifiers

Local EPrints ID: 371818
URI: https://eprints.soton.ac.uk/id/eprint/371818
ISSN: 0903-1936
PURE UUID: e887b33f-5c05-4f0d-99a1-7e7e5414a13f
ORCID for Joy Conway: ORCID iD orcid.org/0000-0001-6464-1526

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Date deposited: 18 Nov 2014 16:56
Last modified: 21 Oct 2018 00:37

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