title: Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency.
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.affiliation: 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, MD, 20892-1960, USA. komarowh@niaid.nih.gov.
authors.forename: Hirsh D
authors.initials: HD
authors.lastname: Komarow
authors.affiliation: National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, 20892, USA. Robert.Sokolic@fda.hhs.gov.
authors.forename: Robert
authors.initials: R
authors.lastname: Sokolic
authors.affiliation: Department of Biochemistry, Duke University School of Medicine, Durham, NC, USA. michael.hershfield@dm.duke.edu.
authors.forename: Michael S
authors.initials: MS
authors.lastname: Hershfield
authors.affiliation: Department of Microbiology, Immunology and Molecular Genetics, University of California Los Angeles, David Geffen School of Medicine, Los Angeles, CA, USA. dkohn@mednet.ucla.edu.
authors.forename: Donald B
authors.initials: DB
authors.lastname: Kohn
authors.affiliation: Clinical Research Directorate/Clinical Monitoring Research Program, Leidos Biomedical Research, Inc., Frederick National Laboratory for Clinical Research, Frederick, MD, 21702, USA. youngmic@mail.nih.gov.
authors.forename: Michael
authors.initials: M
authors.lastname: Young
authors.affiliation: 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, MD, 20892-1960, USA. DMETCALFE@niaid.nih.gov.
authors.forename: Dean D
authors.initials: DD
authors.lastname: Metcalfe
authors.affiliation: National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, 20892, USA. fabio.candotti@chuv.ch.|Division of Immunology and Allergy, University Hospital of Lausanne, CH-1101, Lausanne, Switzerland. fabio.candotti@chuv.ch.
authors.forename: Fabio
authors.initials: F
authors.lastname: Candotti
mesh_terms: D000243:Adenosine Deaminase / Q000172:deficiency*; D000293:Adolescent; D000361:Agammaglobulinemia / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008168:Lung / Q000502:physiology*; D008171:Lung Diseases / Q000175:diagnosis*; D008297:Male; D009991:Oscillometry / Q000379:methods*; D011446:Prospective Studies; D016511:Severe Combined Immunodeficiency / Q000175:diagnosis*; D013147:Spirometry
chemical_list: D000243:Adenosine Deaminase