Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency.
Détails
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Etat: Public
Version: Final published version
Etat: Public
Version: Final published version
ID Serval
serval:BIB_9D71F79E18A7
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency.
Périodique
Orphanet Journal of Rare Diseases
ISSN
1750-1172 (Electronic)
ISSN-L
1750-1172
Statut éditorial
Publié
Date de publication
2015
Peer-reviewed
Oui
Volume
10
Numéro
1
Pages
159
Langue
anglais
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
Publication Status: epublish
Résumé
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.
Mots-clé
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
Oui
Création de la notice
11/01/2016 18:25
Dernière modification de la notice
20/08/2019 16:03