a condition in which a child is born without a thymus gland, resulting in a complete absence of functional T cells. Normal B cell function is present.
Etymology: Angelo M. DiGeorge, American physician, b. 1921
a congenital disorder characterized by severe immunodeficiency and structural abnormalities, including hypertelorism; notched, low-set ears; small mouth; downward slanting eyes; cardiovascular defects; and absence of the thymus and parathyroid glands. Death, often as a result of infection, usually occurs before 2 years of age. Rarely transplantation of a human fetal thymus is performed. Also called thymic hypoplasia, thymic parathyroid aplasia.
an immunodeficiency syndrome in humans and mice, associated with thymic hypoplasia or aplasia and absence of T lymphocytes, resulting from a congenital absence of the third and fourth branchial pouches.