Chronic granulomatous disease.
Chronic granulomatous disease (CGD) was first described in the 1950s and has become a paradigm for genetic neutrophil diseases. It is characterized by recurrent infections with a narrow spectrum of bacteria and fungi as well as a common set of inflammatory complications most notably including inflammatory bowel disease. Over the last half century major advances in management have profoundly altered the major clinical issues and the life expectancy of CGD. With X-linked and autosomal recessive forms, it has been an important disease for the development of bone marrow transplantation and gene therapy. Some of the recent developments in infectious syndromes, inflammatory complications, and curative approaches are discussed in this review.
Holland
Steven M
SM
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Department of Health and Human Services, Bethesda, MD 20892-1684, USA. smh@nih.gov
D000818:Animals; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000935:Antifungal Agents / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D015316:Genetic Therapy; D006105:Granulomatous Disease, Chronic / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000628:therapy; D006801:Humans; D007371:Interferon-gamma / Q000627:therapeutic use; D051379:Mice; D019255:NADPH Oxidases / Q000172:deficiency / Q000235:genetics
D000900:Anti-Bacterial Agents; D000935:Antifungal Agents; D007371:Interferon-gamma; D019255:NADPH Oxidases