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- Title
Special Article: Chronic granulomatous disease in the United Kingdom and Ireland: a comprehensive national patient-based registry.
- Authors
Jones, L. B. K. R.; McGrogan, P.; Flood, T. J.; Gennery, A. R.; Morton, L.; Thrasher, A.; Goldblatt, D.; Parker, L.; Cant, A. J.
- Abstract
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.
- Subjects
ASPERGILLOSIS; CHRONIC granulomatous disease; COLITIS; CROHN'S disease; PNEUMONIA; STAPHYLOCOCCAL diseases; MEDICAL informatics
- Publication
Clinical & Experimental Immunology, 2008, Vol 152, Issue 2, p211
- ISSN
0009-9104
- Publication type
Article
- DOI
10.1111/j.1365-2249.2008.03644.x