Recombinant human interferon-gamma reconstitutes defective phagocyte function in patients with chronic granulomatous disease of childhood.

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Abstract
Monocytes from 19 to 30 patients with the classic phenotype of chronic granulomatous disease of childhood (CGD) responded to 3 days of treatment in culture with recombinant human interferon-.gamma. (rHuIFN-.gamma.) at 100 units/ml by producing superoxide after stimulation with phorbol 12-myristate 13-acetate. Cells from 15 to 16 patients with cytochrome b-positive CGD (15 with autosomal and 1 with X chromosome-liked inheritance) and cells from 4 of 14 patients with cytochrome b-negative CGD (13 with X chromosome-linked and 1 with autosomal recessive inheritance) responded. Subcutaneous rHuIFN-.gamma. (0.01-0.05 mg/m2) administered as a single dose, daily or every other day, for five or six doses to 3 patients whose phagocytes responded to rHuIFN-.gamma. in vitro resulted in significant improvement in phagocyte bactericidal activity against Staphylococcus aureus and increases in superoxide production. Studies on 1 patient''s cells indicated the increases in superoxide production correlated with increased membrane cytochrome b. The effects of rHuIFN-.gamma. persisted for more than a week following cessation of therapy. Thus, we have demonstrated a partial correction in vivo of these CGD patients'' phagocyte defect with rHuIFN-.gamma.. Moreover, the data suggest that a significant proportion of patients with CGD will respond to rHuIFN-.gamma. with augmentation of phagocyte microbicidal function.

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