TAILIEUCHUNG - Modern management of chronic granulomatous disease

An external consultant was also hired to develop tools and carry out computerized analysis of data for the baseline and end-line surveys. An orientation session on data collection was provided to a team of five persons from each of the five target provinces of Thanh Hoa, Quang Ngai, Binh Duong, Dong Nai and Ho Chi Minh, to help them understand the questionnaires and how to fill in data. These surveys reached up to 734 individuals, comprising 416 women with children under five, and 318 day care workers. Results of two surveys were available in late September and late. | bjh review Modern management of chronic granulomatous disease Reinhard A. Seger Division Immunology Haematology University Children s Hospital of Zurich Zurich Switzerland Summary Chronic granulomatous disease CGD is a rare primary immunodeficiency disorder of phagocytic cells resulting in failure to kill a characteristic spectrum of bacteria and fungi and in defective degradation of inflammatory mediators with concomitant granuloma formation. Current prophylaxis with trimethoprim-sulfamethoxazole itraconazole and in selected cases additional interferon gamma is efficient but imperfect. A significant recent progress towards new antibiotic . linezolid and antifungal . voriconazole and posaconazole therapy will allow survival of most patients into adulthood. Adolescent and adult CGD is increasingly characterized by inflammatory complications such as granulomatous lung and inflammatory bowel disease requiring immunosupressive therapy. Allogeneic haematopoietic stem cell transplantation from a human leucocyte antigen identical donor is currently the only proven curative treatment for CGD and can be offered to the selected patients. Gene-replacement therapy for patients lacking a suitable stem cell donor is still experimental and faces major obstacles and risks. However it may offer some transitory benefits and has helped in a few cases to overcome life-threatening infections. Keywords antifungal agents interferon gamma corticosteroids stem cell transplantation gene therapy. Aetiology and pathogenesis of the disease Chronic granulomatous disease CGD is an inherited immunodeficiency disorder which results from the absence or malfunction of NADPH oxidase subunits in phagocytic cells . in neutrophils monocytes macrophages and eosinophils. This oxidase is directly responsible for production of superoxide the so-called respiratory burst converted into microbicidal reactive oxygen species such as hydrogen peroxide hydroxylanion and hypochlorous acid and indirectly .

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