title: Chemically induced congenital thymic dysgenesis in the rat: a model of the DiGeorge syndrome.
abstract: The DiGeorge syndrome, a variable complex of thymic aplasia, congenital heart disease, hypoparathyroidism, and anomalies of the face and neck, is thought to result from exposure to teratogenic agents. A group of congenital defects closely resembling this syndrome can be produced in newborn rats by the administration of the fat-soluble zinc chelating agent WIN 18,446, a bis-dichloroacetylamine. This drug, nontoxic to adult animals, is a powerful teratogen when administered to pregnant rats during days 9-12 of the 21-day gestation period. Our animal data suggest that the human syndrome results from exposure in utero to agents like WIN 18,446, which damage the fetus during a critical period of organogenesis.
mesh_terms: D000818:Animals; D004062:DiGeorge Syndrome / Q000139:chemically induced* / Q000196:embryology / Q000276:immunology; D003959:Diamines / Q000008:administration & dosage; D004195:Disease Models, Animal; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000139:chemically induced*; D011247:Pregnancy; D051381:Rats; D013950:Thymus Gland / Q000196:embryology / Q000473:pathology; D013997:Time Factors
chemical_list: D003959:Diamines; C002491:N,N'-bis(dichloroacetyl)-1,8-octamethylenediamine