Pathophysiology Chronic granulomatous disease

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two neutrophils among many red blood cells. neutrophils 1 type of cell affected chronic granulomatous disease.


phagocytes (i.e. neutrophils , macrophages) require enzyme produce reactive oxygen species destroy bacteria after ingested (phagocytosis), process known respiratory burst. enzyme termed phagocyte nadph oxidase (phox). enzyme oxidizes nadph , reduces molecular oxygen produce superoxide anions, reactive oxygen species. superoxide disproportionated peroxide , molecular oxygen superoxide dismutase. finally, peroxide used myeloperoxidase oxidize chloride ions hypochlorite (the active component of bleach), toxic bacteria. thus, nadph oxidase critical phagocyte killing of bacteria through reactive oxygen species.


(two other mechanisms used phagocytes kill bacteria: nitric oxide , proteases, loss of ros-mediated killing alone sufficient cause chronic granulomatous disease.)


defects in 1 of 4 essential subunits of phagocyte nadph oxidase (phox) can cause cgd of varying severity, dependent on defect. there on 410 known possible defects in phox enzyme complex can lead chronic granulomatous disease.








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