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Special Article: Chronic granulomatous disease in the United Kingdom and Ireland: a comprehensive national patient-based registry

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

Clinical and experimental immunology, 2008-05, Vol.152 (2), p.211-218 [Periódico revisado por pares]

Oxford, UK: Oxford, UK : Blackwell Publishing Ltd

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Título:
Special Article: Chronic granulomatous disease in the United Kingdom and Ireland: a comprehensive national patient-based registry
Autor: Jones, L.B.K.R ; McGrogan, P ; Flood, T.J ; Gennery, A.R ; Morton, L ; Thrasher, A ; Goldblatt, D ; Parker, L ; Cant, A.J
Assuntos: Adolescent ; Adult ; Analytical, structural and metabolic biochemistry ; Aspergillosis - complications ; Aspergillosis - epidemiology ; Aspergillus infection ; Biological and medical sciences ; Child ; Child, Preschool ; chronic granulomatous disease ; colitis ; Epidemiologic Methods ; Female ; Fundamental and applied biological sciences. Psychology ; Granulomatous Disease, Chronic - complications ; Granulomatous Disease, Chronic - epidemiology ; Hematologic and hematopoietic diseases ; Humans ; Immunodeficiencies ; Immunodeficiencies. Immunoglobulinopathies ; Immunopathology ; Infant ; Infant, Newborn ; Ireland - epidemiology ; Male ; Medical sciences ; Middle Aged ; Opportunistic Infections - complications ; Opportunistic Infections - epidemiology ; Other diseases. Hematologic involvement in other diseases ; pneumonia ; Respiratory Tract Infections - complications ; Respiratory Tract Infections - epidemiology ; Special ; Staphylococcal infection ; Staphylococcal Infections - complications ; Staphylococcal Infections - epidemiology ; United Kingdom - epidemiology
É parte de: Clinical and experimental immunology, 2008-05, Vol.152 (2), p.211-218
Notas: http://dx.doi.org/10.1111/j.1365-2249.2008.03644.x
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SourceType-Scholarly Journals-1
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Descrição: 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.
Editor: Oxford, UK: Oxford, UK : Blackwell Publishing Ltd
Idioma: Inglês

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Special Article: Chronic granulomatous disease in the United Kingdom and Ireland: a comprehensive national patient-based registry

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

Oxford, UK: Oxford, UK : Blackwell Publishing Ltd

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