CONGENITALE NEBENNIERENRINDENHYPERPLASIE BEIM MANN MIT NORMALER KEIMDRÜSENFUNKTION UND FERTILITÄT

Abstract

ABSTRACT Brother and sister with congenital hyperplasia of adrenal glands are described. The female, 21 years old, presents a feminine pseudohermaphroditism having before undergone a precocious puberty of the heterosexual type. The male, 33 years old, has had a precocious puberty of isosexual type. His testes are of normal size, the spermiogram permits fertility. He is father of two children. Similar cases of congenital hyperplasia of adrenal glands with and without hypogonadism in male subjects are discussed. We suppose that atrophy of the testes depends on the oestrogen secretion of the adrenals. Over-production of androgens without oestrogens may lead to complete precocious puberty. In this case androgens may cause a precocious maturation of the hypothalamic sexual centre.