Role of Interferon-gamma 1-b (IFN-γ) on Cells of the Innate Immune System: Functional, Biochemical and Gene Expression Studies in Patients With Chronic Granulomatous Disease

Role of Interferon-gamma 1-b (IFN-γ) on Cells of the Innate Immune System: Functional, Biochemical and Gene Expression Studies in Patients With Chronic Granulomatous Disease

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.

Inclusion Criteria:

• Current treatment with IFN-γ or intent to treat with this drug

• Acute infections resolved

• Subject is off treatment medications

• Women of childbearing potential must have:

  • a negative urine beta-HCG, and
  • be willing to practice two forms of effective contraception for the duration of study participation

Exclusion Criteria:

• Pregnancy,

• Breast-feeding, or

• Unwillingness to use effective contraception

• Recent vaccination (within the last fourteen days)

• Current infection or acute health condition requiring treatment

• Chronic inflammatory complications of CGD including:

  • gastric outlet or urinary tract obstruction,
  • pleuritis,
  • pericarditis,
  • inflammatory bowel disease, or
  • colitis requiring treatment.

• Un-associated chronic lung, liver, kidney, or cardiac disorders requiring active treatment