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• W1572163062 abstract "Chronic granulomatous disease (CGD) is a rare primary immunodeficiency syndrome characterized by mutations in one of the four genes that encode the subunits of NADPH oxidase, a enzyme principally involved in the ROS mediated pathogen killing. The patients are characterized by life-threatening and recurrent infections mainly by catalase-positive microorganisms, due to the inability of their phagocytes to express a respiratory burst against invading bacteria (e.g. Staphylococcus aureus) and fungi (e.g. Aspergillus fumigatus and Candida albicans). Patients, also suffer from a variety of sterile inflammatory conditions as acute and/or chronic inflammation with fibrosis containing non-caseous granulomas in the intestinal tract, liver, lymph nodes, urogenital tract, skin, and brain, most probably caused by an intrinsic dysregulation of the inflammatory mechanisms that can involve other cell types than the known defective phagocytes.Although in literature there are few immunological studies about cell function in CGD, a recent work on CGD animal model, has highlighted that in acute fungal infection, a dysregulation of IL-17-secreting cells, coupled with a decrease IFN-γ-producing effector cells and of IL-10-producing regulatory cells, could have a pathogenic role. Moreover, a decreased frequency of a specific T lymphocytes compartment, called Th17, for their characteristic IL-17A production, was associated with increased susceptibility to fungal end bacterial infection in several human chronic disease as hyper IgE syndrome (HIES) and chronic mucocutaneous candidiasis.In this study, we have investigated the potential involvement of T lymphocyte deregulation in CGD manifestations, by comparison of proliferation, mRNA expression, cytokine production and T lymphocyte phenotype upon fungal and bacterial antigen stimulation, of peripheral blood mononuclear cells (PBMC) between a cohort of CGD patients and of healthy controls.Despite a very large variability in functional responses found in both patient and controls, we showed a statistical significant increased of proliferation upon C. albicans lysate and of IL-10 production upon A. fumigatus in CGD patients, coupled with a slight increase of IFN-γ and IL-17A upon fungal stimulation.Furthermore, upon A. fumigatus in the CDG cohort was found a statistically significant increased of IL-17A production, excluded one patient with chronic inflammation, and increased IL-17A mRNA expression, in agreement with an involvement of IL-17A+ cells in CGD pathogenesis, as shown by the CGD animal model of invasive aspergillosis.We also observed that, in agreement with a protective role of IFN-γ, CGD patients that experienced severe aspergillosis expressed low IFN-γ production compared with other patients, but similar to controls, while patient that not experienced severe aspergillosis had increased IFN-γ production compared to controls, demonstrating that a compensatory IFN-γ increased was need to protect patients with defect of ROS-mediated killing. Concerning the responses to C. albicans yeast and hyphae stimulation, we observed similar amount of IFN-γ and increased of IL-17A and IL-10 levels, compared to controls, showing that probably different pathological mechanism lead to increased Candida albicans infections in CGD with respect to HIES, because it would not be dependent on a decrease of IL17A response to fungal antigens.Moreover, in comparison to CGD patients, HIES patients expressed lower value of IFN-γ for all antigens, but differently to STAT3-mutated HIES patient, that not express IL-17A upon all kinds of stimulation, in STAT3-wt HIES patient IL-17A production upon both C. albicans yeast and hyphae was similar to CGD and increased compared to age-matched healthy controls. Thus the reduced frequencies of C. albicans infection in CGD compared to STAT3-wt HIES, may be explained through the main protective level of IFN-γ. Moreover we believe that in vitro immunological test can help us to exclude a STAT3 mutation, in patients who have a positive score for HIES, before performing a molecular analysis of the gene." @default.
• W1572163062 created "2016-06-24" @default.
• W1572163062 creator A5011283417 @default.
• W1572163062 date "2010-06-14" @default.
• W1572163062 modified "2023-09-26" @default.
• W1572163062 title "Adaptive immunity in a human chronic inflammatory disorder: phenotypic and functional characterization of T lymphocytes in a cohort of Chronic Granulomatous Disease patients" @default.
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