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Irish Child Health Database - Peer Reviewed Papers

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  • Special article: chronic granulomatous disease in the United Kingdom and Ireland: a comprehensive national patient-based registry

    Published in:

    Clin Exp Immunol, Vol: 152, Page: 211-8

    Publication Date:

    May 2008

    Aims & Objectives:

    The aim of this study was to examine chronic granulomatous disease in the United Kingdom and Ireland

    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.

    Authors:

    L. B. Jones; P. McGrogan; T. J. Flood; A. R. Gennery; L. Morton; A. Thrasher; D. Goldblatt; L. Parker; A. J. Cant

    Study Type:

    Study Papers » Case Study » Descriptive Studies - Without a comparision

    Categories:

    Chronic granulomatous disease

    International Classification:

    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism - Other diseases of blood and blood-forming organs - Chronic granulomatous disease

    Keywords:


    Geography:

    Republic of Ireland and the United Kingdom (UK & Ireland)