Digeorg syndrome

Children with immunodeficiency syndrome caused by primary T-cell immunodeficiency mainly exhibit cell-mediated immunosuppression, accounting for 5% to 20% of primary immunodeficiency disease. T cell defects also indirectly affect effector cells, such as monocytes and B cells, that are activated by T cells initiated by the antigen. Severe T cell defects produce clinical signs more than one year after birth, which can be manifested as a combined immune deficiency of different immune response systems, and mild T cell defects can appear as late as adulthood. These include thymic hypoplasia, hypocalcemia, congenital heart disease, and facial deformities. Most of the cases are part of Diegger's syndrome, that is, the thymus is not damaged, the number and function of T cells are normal, and infection is rare. A few cases are complete Digeorge syndrome, that is, those with thymic defects. Some of the sick children also have partial thymic defects, and their immune function defects gradually improve over time. The number and function of T cells in patients with complete Diegger syndrome are significantly reduced, and they have a clear tendency to infect.