Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency.
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ABSTRACT:
SUBMITTER: Komarow HD
PROVIDER: S-EPMC4683718 | biostudies-literature | 2015 Dec
REPOSITORIES: biostudies-literature
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Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency.
Komarow Hirsh D HD Sokolic Robert R Hershfield Michael S MS Kohn Donald B DB Young Michael M Metcalfe Dean D DD Candotti Fabio F
Orphanet journal of rare diseases 20151218
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 pro ...[more]
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