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

Détails

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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
Auteur⸱e⸱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
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
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
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