Iba K, Hamada N, Sowa E, Morii H, Wada M
Endocrinol Jpn. 1976 Aug;23(4):289-93.
A case of 20-year-old woman with hypogonadotropic hypogonadism and anosmia is reported, since very few female cases of Kallmann's syndrome have been reported so far in Japan. Three uncles on the father's side had no children. Height was 168 cm, and arm span 165 cm. The olfactory test revealed complete anosmia. Bone age was 13 year. Chromosome was 46 XX and normal karyotype. Basal levels of serum FSH, LH and estrogens (E1, E2 and E3) were low. Serum FSH and LH levels rose slightly only after LH-RH administration, and did not increase in clomiphene test. Plasma estrogens did not increase after daily injection of 150 IU of HMG for 3 successive days. The response of serum GH to arginine infusion was normal, while that to insulin-induced hypoglycemia was poor.
本文报告了一例20岁患有低促性腺激素性性腺功能减退和嗅觉缺失的女性病例，因为目前在日本报道的卡尔曼综合征女性病例非常少。父亲一方的三位叔叔没有子女。身高168厘米，臂展165厘米。嗅觉测试显示完全嗅觉缺失。骨龄为13岁。染色体为46，XX，核型正常。血清促卵泡生成素（FSH）、促黄体生成素（LH）和雌激素（E1、E2和E3）的基础水平较低。仅在注射促黄体生成素释放激素（LH-RH）后，血清FSH和LH水平略有上升，而在氯米芬试验中未升高。连续3天每日注射150国际单位人绝经期促性腺激素（HMG）后，血浆雌激素未增加。血清生长激素（GH）对精氨酸输注的反应正常，而对胰岛素诱导的低血糖反应较差。
