Title |
Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency
|
---|---|
Published in |
Orphanet Journal of Rare Diseases, December 2015
|
DOI | 10.1186/s13023-015-0365-z |
Pubmed ID | |
Authors |
Hirsh D. Komarow, Robert Sokolic, Michael S. Hershfield, Donald B. Kohn, Michael Young, Dean D. Metcalfe, Fabio Candotti |
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. |
X Demographics
Geographical breakdown
Country | Count | As % |
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Finland | 1 | 14% |
United Kingdom | 1 | 14% |
Unknown | 5 | 71% |
Demographic breakdown
Type | Count | As % |
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Scientists | 4 | 57% |
Members of the public | 3 | 43% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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Unknown | 15 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Master | 6 | 40% |
Other | 2 | 13% |
Student > Postgraduate | 2 | 13% |
Researcher | 1 | 7% |
Librarian | 1 | 7% |
Other | 0 | 0% |
Unknown | 3 | 20% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 6 | 40% |
Computer Science | 2 | 13% |
Chemical Engineering | 1 | 7% |
Social Sciences | 1 | 7% |
Immunology and Microbiology | 1 | 7% |
Other | 0 | 0% |
Unknown | 4 | 27% |