[Hormonal principles in normal and pathologic somatic sexual development]

Abstract

Normal sexual development is the consequence of three sequential interrelated processes: establishment of genetic, gonadal and somatic sex. It is the terminal phase of sexual differentiation--the translation of gonadal into somatic sex, which is governed by the presence or absence of both testosterone and Müllerian-inhibiting hormone and of dihydrotestosterone, which is formed in its respective target tissues. Thus, despite a testis, somatic male sexual differentiation will proceed to a normal male phenotype only if all three hormones are synthesized and act during a critical period of uterine development. Many clinically distinct syndromes are the results of abnormalities in the synthesis or action of the above-mentioned hormones; these syndromes are described in detail. In contrast to male somatic differentiation, female somatic development is independent of these hormones.