Chronic granulomatous disease (CGD) is a primary immunodeficiency resulting from the absence or malfunction of oxidative mechanism in phagocytic cells. The disease is due to a mutation in one of four genes that encode subunits of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Affected patients experience severe infections and granuloma formation due to exuberant inflammatory responses. Some evidence suggests that eosinophilic cystitis (EC) is included in the spectrum of inflammatory manifestations. EC is an inflammatory disease, rare in childhood, which may require different, nonstandardized therapeutic approaches, ranging from antihistamines to cyclosporine.
Claps, A., Della Corte, M., Gerocarni Nappo, S., Francalanci, P., Palma, P., Finocchi, A. (2014). How should eosinophilic cystitis be treated in patients with chronic granulomatous disease?. PEDIATRIC NEPHROLOGY, 29(11), 2229-2233 [10.1007/s00467-014-2883-7].
How should eosinophilic cystitis be treated in patients with chronic granulomatous disease?
Palma, P;FINOCCHI, ANDREA
2014-11-01
Abstract
Chronic granulomatous disease (CGD) is a primary immunodeficiency resulting from the absence or malfunction of oxidative mechanism in phagocytic cells. The disease is due to a mutation in one of four genes that encode subunits of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Affected patients experience severe infections and granuloma formation due to exuberant inflammatory responses. Some evidence suggests that eosinophilic cystitis (EC) is included in the spectrum of inflammatory manifestations. EC is an inflammatory disease, rare in childhood, which may require different, nonstandardized therapeutic approaches, ranging from antihistamines to cyclosporine.File | Dimensione | Formato | |
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