Fatal Granulomatous Disease of Childhood

NADPH oxidase deficiency in X-linked chronic granulomatous disease.

“…Although neutrophils (PAMN) from patients with CGD ingest microorganisms effectively, they fail to kill many bacteria and fungi at a normal rate (3,4). Phagocytosis by nornmal PAMN is associated with major changes in oxidative metabolism, including increased cyanide-insensitive consumption of oxygen (5)(6)(7), stimulation of glucose oxidation via the hexose monophosphate shunt (HMAPS) (7,8), increased generation of hydrogen peroxide and superoxide (9)(10)(11), and augmented reduction of the redox dye, nitroblue tetrazoliunm (NBT) This work was presented in part at the National Meeting of the American Federation for Clinical Research, Atlantic City, N. J., May, 1974, and published in abstract form (1).…”

Leukocyte degranulation and vacuole formation in patients with chronic granulomatous disease of childhood

“…The polymorphonuclear leukocyte (PMN) of these patients has been shown to have decreased bactericidal activity by in vitro measurement (2). Recent reports have focused attention on morphologic and metabolic abnormalities in the functionally abnormal PMN.…”

Chronic Granulomatous Disease: Mode of Action of Sulfamethoxazole/Trimethoprim

“…This could explain the penicillin resistance of phagocytosed bacteria reported by Holrnes el al. (15) in 1966.…”