Chronic Septic Granulomatosis in Children
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47,99 |
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Beschrijving
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Chronic septic granulomatosis is a rare primary immunodeficiency caused by a dysfunction of the NADPH oxidase complex in phagocytes.The disease is genetic in origin, most commonly inherited in an X-linked recessive pattern, and less commonly in an autosomal recessive pattern. X-linked forms are generally more severe and manifest at an earlier age.The diagnosis is often made in the first few years of life due to severe and recurrent infections. Patients are particularly susceptible to bacterial and fungal infections, especially invasive aspergillosis.Chronic inflammatory granulomas can also affect multiple organs and impair the quality of life of affected children.
Beschrijving
(1)
Chronic septic granulomatosis is a rare primary immunodeficiency caused by a dysfunction of the NADPH oxidase complex in phagocytes.The disease is genetic in origin, most commonly inherited in an X-linked recessive pattern, and less commonly in an autosomal recessive pattern. X-linked forms are generally more severe and manifest at an earlier age.The diagnosis is often made in the first few years of life due to severe and recurrent infections. Patients are particularly susceptible to bacterial and fungal infections, especially invasive aspergillosis.Chronic inflammatory granulomas can also affect multiple organs and impair the quality of life of affected children.
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