Abstract
CGD is characterized by deficient membrane bound NADPH-oxidase activity in phagocytes. These patients suffer from severe recurrent bacterial and fungal infections. In a boy with typical clinical history of CGD, investigations of his total leukocytes showed reduced cytochemical nitrobluetetrazolium (NBT)-reduction (30-48 % NBT-positive phagocytes), decreased O2−-production in response to the phorbolester PMA and low glucose oxidation. Because in typical CGD-patients at least 99 % of the phagocytes are NBT-negative, we suspected a variant of CGD. Extensive examinations in pure (> 98 %) preparations of polymorphonuclear leukocytes (PMN) gave results compatible with classical CGD: absent production of chemiluminescence and oxygen metabolites (O2−, H2O2) after stimulation with PMA and opsonized zymosan, low glucose oxidation, decreased bactericidal capacity and 99 % NBT-negative PMN. However, in monocytes of the patient, a nearly normal PMA-induced O2−-formation has been found (11.4 nmole O2−/h/2×105 cells, controls 18.2±2.3). The same was true with H2O2-production. In addition the patient's macrophages, cultivated in a long term culture, similarly released oxygen metabolites after stimulation of the respiratory burst with either PMA of opsonized zymosan. We demonstrate here an isolated defect of the NADPH-oxidase activity in neutrophils of a patient with CGD.
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Gahr, M., Allgeier, B., Seibring, A. et al. CHRONIC GRANULOMATOUS DISEASE (CGD): ISOLATED DEFECT OF THE NADPH-OXIDASE ACTIVITY IN NEUTROPHILS. Pediatr Res 22, 224 (1987). https://doi.org/10.1203/00006450-198708000-00063
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DOI: https://doi.org/10.1203/00006450-198708000-00063