Di George syndrome. Definition

Medical Definition: Di George syndrome

Rare congenital disorder due to the dysmorphogenesis of the third and fourth pharyngeal pouch, leading to hypoplasia or aplasia of the thymus and parathyroid glands and abnormal development of other structures formed at the same age. The causes abnormal development of parathyroid tetany and hypocalcemia, while thymic hypoplasia, immunodeficiency disease with T cells with normal maturation of progenitor cells and B lymphocytes For this reason, patients with this syndrome have normal concentrations of immunoglobulins and an increased susceptibility to opportunistic infections, as well as graft versus host disease due to blood transfusions. Does not require treatment, because the T cell function tends to improve with age, becoming normal to five years, probably due to the functional ExtraTime locations ripe for T lymphocytes, characteristic of the thymus.

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