真两性畸形：从女性内分泌状态到男性内分泌状态。

True hermaphroditism: from female to male endocrine status.

作者信息

Kuhn J M, Cleret J M, Lavoinne A, Laudat M H, Hemet J, Wolf L M

出版信息

J Clin Endocrinol Metab. 1985 Jul;61(1):196-9. doi: 10.1210/jcem-61-1-196.

Abstract

True hermaphroditism was revealed by monthly intrascrotal bleeding in a 21-yr-old subject of male phenotype who had undergone surgical treatment for gonadal ectopy at the age of 7 yr. The presence of an ovary was demonstrated by the endocrine profile of an ovulatory menstrual cycle. Evidence for the presence of a testis was provided by a plasma testosterone increase after hCG administration (5000 IU/day for 3 days) and its spontaneous response to an endogenous preovulatory LH peak. Further endocrine studies revealed that both gonads were stimulated by endogenous gonadotropins. At surgery, a hemiuterus and an ovary with corpus luteum were found in the left hemiscrotum, and a testis and epididymis were found in the right hemiscrotum. After removal of the ovary, the subject passed from a predominantly female to a male endocrine status, which suggests that the endocrine secretion of the testis was inhibited by the negative feedback effect of ovarian steroids on gonadotropin secretion.

摘要

一名21岁男性表型患者，7岁时因性腺异位接受手术治疗，每月阴囊内出血揭示了真两性畸形。排卵性月经周期的内分泌特征证实了卵巢的存在。人绒毛膜促性腺激素（5000 IU/天，共3天）给药后血浆睾酮升高，以及其对内源性排卵前促黄体生成素峰值的自发反应，为睾丸的存在提供了证据。进一步的内分泌研究表明，两个性腺均受内源性促性腺激素刺激。手术时，在左侧半阴囊中发现一个半子宫和一个有黄体的卵巢，在右侧半阴囊中发现一个睾丸和附睾。切除卵巢后，该患者从主要为女性内分泌状态转变为男性内分泌状态，这表明卵巢类固醇对促性腺激素分泌的负反馈作用抑制了睾丸的内分泌分泌。