Correlation of Metabolic and Chemiluminescent Responses of Granulocytes from Three Female Siblings with Chronic Granulomatous Disease

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Abstract
Polymorphonuclear leukocytes (PMNL) from children with atypical chronic granulomatous disease (CGD), their mother and siblings, and normal controls were studied in regard to glycolytic and hexose monophosphate shunt activities in the resting, methylene blue-stimulated, and phagocytizing states. PMNL from the patients with CGD had normal glycolytic and hexose monophosphate shunt activities in the resting state and after stimulation with methylene blue. However, stimulation of the hexose monophosphate shunt after phagocytosis was greatly decreased. These data were correlated with studies of both initial rate and integral counts of chemiluminescence. The chemiluminescent response from patients with atypical CGD was also greatly decreased. This decreased response probably reflects a defect in the oxidative destruction of the phagocytized microbe and correlates well with the decreased activity of the phagocytically activated hexose monophosphate shunt. The defect in generation of radical species of oxygen, singlet oxygen, and chemiluminescence by leukocytes from patients with CGD is discussed.