Defective Superoxide Production by Granulocytes from Patients with Chronic Granulomatous Disease

Abstract
Superoxide-dependent cytochrome c reduction was used to measure Superoxide production by granulocytes from two patients with chronic granulomatous disease, from children of similar age with infectious and noninfectious diseases unrelated to defects in granulocyte metabolism, and from normal adults. Superoxide production by granulocytes from normal adults was 12.4 ± 4.9 (± S.D.) nmoles per 3 x 106 cells per 30 minutes for resting cells, and 29.7 ± 2.8 nmoles for cells incubated with bacteria (stimulated cells). Values of 10.9 ± 1.5 and 26.1 ± 3.1 nmoles for resting and stimulated granulocytes obtained from children 11 to 18 months of age were not significantly different from corresponding values in adult granulocytes. Superoxide production by cells from two patients with chronic granulomatous disease was greatly reduced (0.3 and 0.2 for resting cells, and 0.9 and 0.9 for stimulated cells), but normal production was obtained with cells from both parents of one of these patients and from the mother of the second patient. (N Engl J Med 290:593–597, 1974)