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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


    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.


    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


    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



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