Due to decreased maturation numbers and dysfunctional peripheral T cells, infants born with improper thymus development (caused by DiGeorge syndrome or FoxN1 mutations) experience life-threatening infections that cause early mortality during the first year of life 5-7.
Prevention and Mitigation.
DiGeorge syndrome (DGS) is a primary maturation immunodeficiency disease (PIDD) characterised by impaired thymocyte development and function, which increases vulnerability to infections. T-cells are taught in the thymus how to combat infection and stave off autoimmune disease. Primary immunodeficiency known as DiGeorge Syndrome (DGS) is characterised by cellular (T-cell) deficit, distinctive facial features, congenital cardiac disease, and hypocalcemia.Due to a comparative decrease in naive CD4+ T-cells, patients with DiGeorge syndrome have high memory CD4+ T-cell counts. Low absolute T cell lymphopenia is frequent in our sample of DiGeorge syndrome individuals, and it gets less severe with age.
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