Chronic granulomatous disease: why an inflammatory disease?

Chronic granulomatous disease is a primary immunodeficiency caused by mutations in the genes encoding subunits of the phagocytic NADPH oxidase system. Patients can present with severe, recurrent infections and noninfectious conditions. Among the latter, inflammatory manifestations are predominant, e...

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Veröffentlicht in:Brazilian journal of medical and biological research 2014-11, Vol.47 (11), p.924-928
Hauptverfasser: Simao, H.M.L, Roxo-Junior, P
Format: Artikel
Sprache:eng
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Zusammenfassung:Chronic granulomatous disease is a primary immunodeficiency caused by mutations in the genes encoding subunits of the phagocytic NADPH oxidase system. Patients can present with severe, recurrent infections and noninfectious conditions. Among the latter, inflammatory manifestations are predominant, especially granulomas and colitis. In this article, we systematically review the possible mechanisms of hyperinflammation in this rare primary immunodeficiency condition and their correlations with clinical aspects.
ISSN:0100-879X
1414-431X
1414-431X
DOI:10.1590/1414-431X20143735