Interferon-gamma in the management of chronic granulomatous disease.
Bactericidal activity of phagocytic cells depends largely upon their production of highly reactive compounds via the metabolism of oxygen. A lesion anywhere in the biochemical pathway of hydrogen peroxide production potentially can cause chronic granulomatous disease (CGD). Recent work has shown that CGD results from specific abnormalities in the nicotinamide-adenine dinucleotide phosphate (NADPH) system, which includes membrane-associated proteins, NADPH, cytochrome b-558, and several cytosolic proteins. Pharmacologic alteration of phagocytic oxidative metabolism can now be achieved through use of recombinant interferon-gamma (IFN-gamma). Data from a multicenter clinical trial indicate that sustained administration of IFN-gamma is effective in the management of CGD; for patients who received IFN-gamma, a 72% reduction in the relative risk of serious infection was noted in comparison with the risk for patients who received placebo. IFN-gamma reduced not only the number of serious primary infections but also the length of hospitalizations.
Gallin
J I
JI
Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892.
D001424:Bacterial Infections / Q000517:prevention & control*; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000235:genetics / Q000276:immunology; D006801:Humans; D007371:Interferon-gamma / Q000627:therapeutic use*; D010587:Phagocytosis; D011994:Recombinant Proteins
D011994:Recombinant Proteins; D007371:Interferon-gamma