Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency.

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Version: Final published version
Serval ID
serval:BIB_9D71F79E18A7
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency.
Journal
Orphanet Journal of Rare Diseases
Author(s)
Komarow H.D., Sokolic R., Hershfield M.S., Kohn D.B., Young M., Metcalfe D.D., Candotti F.
ISSN
1750-1172 (Electronic)
ISSN-L
1750-1172
Publication state
Published
Issued date
2015
Peer-reviewed
Oui
Volume
10
Number
1
Pages
159
Language
english
Notes
Publication types: Letter ; Research Support, N.I.H., Extramural ; Research Support, N.I.H., Intramural ; Research Support, Non-U.S. Gov't
Publication Status: epublish
Abstract
Adenosine deaminase-deficient severe combined immunodeficiency (ADA-SCID) is characterized by impaired T-, B- and NK-cell function. Affected children, in addition to early onset of infections, manifest non-immunologic symptoms including pulmonary dysfunction likely attributable to elevated systemic adenosine levels. Lung disease assessment has primarily employed repetitive radiography and effort-dependent functional studies. Through impulse oscillometry (IOS), which is effort-independent, we prospectively obtained objective measures of lung dysfunction in 10 children with ADA-SCID. These results support the use of IOS in the identification and monitoring of lung function abnormalities in children with primary immunodeficiencies.
Keywords
Adenosine Deaminase/deficiency, Adolescent, Agammaglobulinemia/diagnosis, Child, Child, Preschool, Female, Humans, Lung/physiology, Lung Diseases/diagnosis, Male, Oscillometry/methods, Prospective Studies, Severe Combined Immunodeficiency/diagnosis, Spirometry
Pubmed
Web of science
Open Access
Yes
Create date
11/01/2016 17:25
Last modification date
20/08/2019 15:03
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