Hereditary urogenital adysplasia: families with recurrence of uterine and renal malformations.

In 1980, Schimke and King introduced the term hereditary urogenital adysplasia to describe recurrence of kidney and uterine malformations which had been observed in several families. They suggested an autosomal dominant inheritance with variable expressivity and penetrance. Now, 35 years later, research is focused towards understanding the underlying genetic causes of this condition. We review the embryonic development of Müllerian and Wolffian ducts and the genes that have been involved in uterine malformations, congenital anomalies of kidney and urinary tract and hereditary urogenital adysplasia in humans.