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Lookup NU author(s): Dr Laura Jones,
Dr Paraic McGrogan,
Dr Terence Flood,
Professor Andrew GenneryORCiD,
Professor Louise Parker,
Professor Andrew Cant
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There are no epidemiological studies from the British Isles of chronic granulomatous disease, characterized by recurrent, life-threatening bacterial and fungal infections and inflammatory sequelae. Patients were enrolled in a national registry and medical records were analysed. Of 94 subjects, 69 had X-linked disease, 16 had autosomal recessive disease and nine were unknown. Prevalence was 7.5/million for 1990-99 and 8.5/million for 1980-89. Suppurative adenitis, abscesses and pneumonia presented commonly. Twenty-three of 30 patients who underwent high resolution computerized tomography had chronic respiratory disease. Inflammatory sequelae included bowel stricture and urogenital tract granulomata. Growth failure was common; 75% of those measured were below the population mean. All patients received prophylactic antibiotics and 93% anti-fungal prophylaxis. Interferon gamma was used to treat infection, but rarely as prophylaxis. Despite prophylaxis, estimated survival was 88% at 10 years but 55% at age 30 years. Morbidity remains significant, severe infectious complications common. Curative treatments including stem cell transplantation should be considered for patients with frequent or serious complications. © 2008 The Author(s).
Author(s): Jones LBKR, McGrogan P, Flood TJ, Gennery AR, Morton L, Thrasher A, Goldblatt D, Parker L, Cant AJ
Publication type: Article
Publication status: Published
Journal: Clinical and Experimental Immunology
Print publication date: 01/05/2008
ISSN (print): 0009-9104
ISSN (electronic): 1365-2249
Publisher: Wiley-Blackwell Publishing Ltd.
PubMed id: 18410635
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