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

Orphanet Journal of Rare Diseases

Fabio Candotti⁵  Dean D. Metcalfe⁴  Michael Young¹  Donald B. Kohn⁶  Michael S. Hershfield²  Robert Sokolic³  Hirsh D. Komarow⁴ 

[1] Clinical Research Directorate/Clinical Monitoring Research Program, Leidos Biomedical Research, Inc., Frederick National Laboratory for Clinical Research, Frederick 21702, MD, USA;Department of Biochemistry, Duke University School of Medicine, Durham, NC, USA;National Human Genome Research Institute, National Institutes of Health, Bethesda 20892, MD, USA;Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, NIH/NIAID/LAD/Bldg. 10, Room 1C129A1, 10 Center Drive, Bethesda 20892-1960, MD, USA;Division of Immunology and Allergy, University Hospital of Lausanne, Lausanne, CH-1101, Switzerland;Department of Microbiology, Immunology and Molecular Genetics, University of California Los Angeles, David Geffen School of Medicine, Los Angeles, CA, USA

关键词: Spirometry;    Impulse oscillometry;    Pulmonary dysfunction;    Children;    SCID;    Adenosine deaminase deficiency;   

Others  :  1235015
DOI  :  10.1186/s13023-015-0365-z

 received in 2015-08-21, accepted in 2015-11-12,  发布年份 2015

【 摘 要 】

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.

【 授权许可】

   
2015 Komarow et al.

【 图 表 】

Fig. 1.

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