Chronic Granulomatous Disease in Females

Leukocyte glutathione peroxidase activity of two female patients with chronic granulomatous disease was shown to be significantly diminished as compared to the activity of control leukocytes. By contrast, the activity of this enzyme in the leukocytes from male patients with the same disease was within normal limits. The activity of glutathione reductase and glucose-6-phosphate dehydrogenase was normal in the same leukocyte extracts from female patients. The demonstration of a deficiency of leukocyte glutathione peroxidase in females distinguishes their disease from that of male patients with the x-linked defect.