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Pathology - Chronic Granulomatous Disease
Chronic granulomatous disease (CGD) is an X-linked recessive condition. Like with other autoimmune disorders, the sorts of microorganisms that infect the patient hint to the deficiency in the immune response. A patient with CGD will have recurrent infections from fungus and staphylococcus (gram-positive cocci in clusters). The deficiency in CGD is inadequate nicotine adenine dinucleotide phosphate oxidase in neutrophil and monocyte cell membranes, resulting to suppressed superoxide dismutase activity and lowered hydrogen peroxide levels. This limits free-radical generation and resulting in an absence respiratory burst, such that phagocytized catalase-positive bacteria are not destroyed. The diagnosis of CGD can be made by the laboratory observation of neutrophils and macrophages failing to decrease a nitroblue tetrazolium dye (leukocytes with an intact respiratory burst create a blue color upon incubation). Myeloperoxidase deficiency, which is a more common abnormality, is characterized by a normal respiratory burst but absence of hypochlorous acid (bleach) synthesis. The only clinical importance, however, shows in vulnerable patients with DM through significant candidal infections.
Chronic granulomatous disease (CGD) is an X-linked recessive condition. Like with other autoimmune disorders, the sorts of microorganisms that infect the patient hint to the deficiency in the immune response. A patient with CGD will have recurrent infections from fungus and staphylococcus (gram-positive cocci in clusters). The deficiency in CGD is inadequate nicotine adenine dinucleotide phosphate oxidase in neutrophil and monocyte cell membranes, resulting to suppressed superoxide dismutase activity and lowered hydrogen peroxide levels. This limits free-radical generation and resulting in an absence respiratory burst, such that phagocytized catalase-positive bacteria are not destroyed. The diagnosis of CGD can be made by the laboratory observation of neutrophils and macrophages failing to decrease a nitroblue tetrazolium dye (leukocytes with an intact respiratory burst create a blue color upon incubation). Myeloperoxidase deficiency, which is a more common abnormality, is characterized by a normal respiratory burst but absence of hypochlorous acid (bleach) synthesis. The only clinical importance, however, shows in vulnerable patients with DM through significant candidal infections.
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