Rappaport R, Nihoul-Fekete C
Ann Endocrinol (Paris). 1980 Jul-Aug;41(4):345-53.
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.
女性假两性畸形最常见的原因是胎儿早期生活中存在雄激素，如先天性肾上腺皮质增生症所见，或孕妇接受激素治疗。本报告回顾了121例病例。记录了对内生殖器进行生殖道造影评估的重要性，主要是泌尿生殖窦。胎儿男性化的发生早在妊娠第9周就因雄激素产生。这个过程很可能依赖促肾上腺皮质激素，然而在肾上腺活动的这个非常早期阶段，人绒毛膜促性腺激素的作用可能仍然存在。有趣的是，肾上腺雄激素不能稳定中肾管衍生物。苗勒管衍生物以及卵巢发育正常。在少数情况下，孕激素已被证明会使胎儿男性化，可能是通过干扰3β-羟类固醇脱氢酶系统。这些药物还与大量非特异性畸形有关。
