Chronic granulomatous disease
Chapter: 2
A group of congenital disorders that can be traced back to defects in the genes that encode the several components of NADPH oxidase, which is critical to the generation of superoxide anion and, therefore, the generation of oxygen-dependent bactericidal mechanisms. Children with this defect are highly susceptible to persistent bacterial infection (especially Gram-positive cocci), because, without benefit of oxygen-dependent killing mechanisms, neutrophils (especially) and other leukocytes are poorly bactericidal. To compensate, the inflammatory response becomes chronic and, finally, granulomatous. (see separately, H2O2-MPO-halide system)
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