Chronic granulomatous disease: Pathogenesis and therapy of associated fungal infections [Infections fongiques et granulomatose septique chronique de la physiopathologie aux nouvelles perspectives thérapeutiques]
Desjardins A.,Center dInfectiologie Necker Pasteur |
Desjardins A.,University of Quebec |
Coignard-Biehler H.,Center dInfectiologie Necker Pasteur |
Coignard-Biehler H.,Center National Of Reference Pour Les Maladies Hereditaires Ceredih |
And 18 more authors.
Chronic granulomatous disease is a genetic disorder responsible for a defect in the NADPH oxidase of phagocytic cells. It impairs the oxidative burst necessary to the intracellular inactivation of microorganisms and predisposes to an increased risk of infections by various microorganisms, including fungi like Aspergillus spp. and other less frequently encountered or emerging fungal species. Here we review the genetic basis, pathogenesis and clinical presentation associated with fungal infections in chronic granulomatous disease as well as the current prophylaxis and newly available therapies. Source