Pseudo-hermaphrodisme féminin. Etiologie et physiopathologie.
Female pseudo-hermaphroditism is due, most frequently, to the presence of androgens during early fetal life as observed in congenital adrenal hyperplasia, or hormone administration to pregnant patients. In this report 121 cases are reviewed. The importance of a genitographic assessment of the internal genitalia, principally the urogenital sinus is documented. The occurrence of fetal masculinisation is due to androgen production as early as the 9th week of gestation. This process is very likely ACTH dependant, however the role of HCG still remains likely at this very early stage of adrenal activity. It is of interest that adrenal androgens are not capable of stabilizing the Wolfian derivatives. Mullerian derivatives develop normally, as well as ovaries. Progestins have been shown to masculinize the fetus in a few cases, probably by interfering with the 3 beta hydroxysteroid deshydrogenase system. The same agents are associated with a large number of non specific malformations.
D000312:Adrenal Hyperplasia, Congenital / Q000150:complications; D012734:Disorders of Sex Development / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D012739:Gonadal Steroid Hormones / Q000009:adverse effects / Q000502:physiology; D006801:Humans; D008431:Maternal-Fetal Exchange; D011247:Pregnancy
D012739:Gonadal Steroid Hormones