Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency
Year: 2015
Type: article
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.
Authors: Hirsh D. Komarow, Robert Sokolic, Michael S. Hershfield, Donald B. Kohn, Michael Young, Dean D. Metcalfe, Fabio Candotti
Institutions: National Institutes of Health, National Institute of Allergy and Infectious Diseases, National Human Genome Research Institute, Duke University, Duke Medical Center, University of California, Los Angeles, Leidos Biomedical Research Inc. (United States), Centre Hospitalier Universitaire Vaudois
Language: English
Subfield: Immunology
Field: Immunology and Microbiology
Domain: Life Sciences
Open Access status: gold
Estimated APC paid: $2,990
Funders: U.S. Department of Health and Human Services, Université de Lausanne, Centre Hospitalier Universitaire Vaudois, National Institutes of Health, National Human Genome Research Institute, National Cancer Institute, National Institute of Allergy and Infectious Diseases, NHLBI Division of Intramural Research
OpenAlex ID: W2211192176