The overall goal of the study is to investigate the functional, biochemical, and gene expression effects of Interferon-gamma 1-b (IFN-γ) on the neutrophils of patients with Chronic Granulomatous Disease (CGD). The investigators hypothesize that the clinical effects demonstrated in patients with CGD treated with IFN-γ (decreased number and severity of infections) are the result of biochemical processes and upregulation of specific genes, which lead to enhanced functionality of this immune cell population.
Study Type
OBSERVATIONAL
Enrollment
9
Study function and biochemistry of neutrophils off the drug for one week and then 12 hours after first dose and fourth dose.
University of Colorado Anschutz Medical Campus
Aurora, Colorado, United States
Changes in Neutrophil bacterialcidal activity in patients with Chronic Granulomatous Disease (CGD) on standard treatment schedule and dose of IFN-γ in comparison to results for the patients off IFN-γ.
Determine changes in neutrophil function in neutrophils from patients with CGD
Time frame: 1 week off drug, 12 hours after a week of treatment
Changes in Gene expression as measured with RNA and affimetrics gene chips in patients with Chronic Granulomatous Disease (CGD) on standard treatment schedule and dose of IFN-γ in comparison to results for the patients off IFN-γ.
Determine changes in gene expression in neutrophils from patients with CGD.
Time frame: 1 week off drug, 12 hours after a week of treatment
Differences in Neutrophil biochemical studies in patients with Chronic Granulomatous Disease (CGD) on standard treatment schedule and dose of IFN-γ in comparison to results for the patients off IFN-γ.
Determine changes in biochemical tests in neutrophils from CGD patients.
Time frame: 1 week off drug, 12 hours after first dose of IFN-gamma; 12 hours after a week of treatment
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