Chronic granulomatous disease (CGD), also known as Bridges–Good syndrome, chronic granulomatous disorder, and Quie syndrome,[1] is a diverse group of hereditary diseases in which certain cells of the immune system have difficulty forming the reactive oxygen compounds (most importantly the superoxide radical due to defective phagocyte NADPH oxidase) used to kill certain ingested pathogens.[2] This leads to the formation of granulomas in many organs.[3] CGD affects about 1 in 200,000 people in the United States, with about 20 new cases diagnosed each year.[4][5]
This condition was first discovered in 1950 in a series of four boys from Minnesota, and in 1957 it was named "a fatal granulomatosus of childhood" in a publication describing their disease.[6][7] The underlying cellular mechanism that causes chronic granulomatous disease was discovered in 1967, and research since that time has further elucidated the molecular mechanisms underlying the disease.[8] Bernard Babior made key contributions in linking the defect of superoxide production of white blood cells, to the cause of the disease. In 1986, the X-linked form of CGD was the first disease for which positional cloning was used to identify the underlying genetic mutation.
^Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
^Heyworth PG, Cross AR, Curnutte JT (October 2003). "Chronic granulomatous disease". Current Opinion in Immunology. 15 (5): 578–84. doi:10.1016/S0952-7915(03)00109-2. PMID 14499268.
^Pao M, Wiggs EA, Anastacio MM, Hyun J, DeCarlo ES, Miller JT, et al. (2004). "Cognitive function in patients with chronic granulomatous disease: a preliminary report". Psychosomatics. 45 (3): 230–4. doi:10.1176/appi.psy.45.3.230. PMID 15123849.
^James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 978-0-7216-2921-6.
^Berendes H, Bridges RA, Good RA (May 1957). "A fatal granulomatosus of childhood: the clinical study of a new syndrome". Minnesota Medicine. 40 (5): 309–12. PMID 13430573.
^Bridges RA, Berendes H, Good RA (April 1959). "A fatal granulomatous disease of childhood; the clinical, pathological, and laboratory features of a new syndrome". A.M.A. Journal of Diseases of Children. 97 (4): 387–408. doi:10.1001/archpedi.1959.02070010389004. PMID 13636694.
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(or chronicgranulomatousdisease (CGD) test) is a measure of neutrophil oxidation and is a useful assay in the diagnosis of chronicgranulomatous disease...
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hypertension Idiopathic chronic fatigue Granulomatous prostatitis Advances in medical science improve the understanding of causes of diseases and the classification...
activating proteins, cause the severe immunodeficiency disease of ChronicGranulomatousDisease (CGD). He showed that the mechanism by which microbes are...
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