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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:
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:
- Adolescent
- Adult
- Aspergillosis/complications/epidemiology
- Child
- Child, Preschool
- Epidemiologic Methods
- Female
- Granulomatous Disease, Chronic/complications/ epidemiology
- Great Britain/epidemiology
- Humans
- Infant
- Infant, Newborn
- Ireland/epidemiology
- Male
- Middle Aged
- Opportunistic Infections/complications/epidemiology
- Respiratory Tract Infections/complications/epidemiology
- Staphylococcal Infections/complications/epidemiology
Geography:
Republic of Ireland and the United Kingdom (UK & Ireland)



