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作者研究了5名男性和3名女性Prader-Willi综合征患者的下丘脑,垂体和性腺的功能。8名患者临床表现均为性腺机能不足,男性病人血液中睾酮均低,然而2名女性病人,在月经周期的卵泡期早期血浆基础雌二醇是在正常范围内,在7名病人中,其基础促性腹激素水平较低,对静脉注射LHRH的反应也低于正常。病人每日口服克罗米酚(Clomiphene)200mg 10天或6周后,再应用LHRH,5个病人中有4个病人LH和FSH升高。5个男性病人全部进行了hCG试验,其中4个病人血浆睾酮的升高低于正常,这4个病人用bCG连续治疗6周,仅有1人睾酮水平暂时上升到正常
The authors studied the hypothalamic, pituitary and gonadal functions in 5 male and 3 female patients with Prader-Willi Syndrome. Eight patients had clinical manifestations of hypogonadism and low testosterone levels in the blood of male patients. However, in two female patients, plasma basal estradiol was in the normal range during the follicular phase of the menstrual cycle and in seven patients, Basal gonadotropin levels are lower and response to intravenous LHRH is also lower than normal. Patients were given oral Clomiphene 200 mg daily for 10 days or 6 weeks before LHRH was applied. Four out of five patients had elevated LH and FSH. All five male patients underwent hCG testing, with four of the patients having an elevated plasma testosterone level below normal. The four patients were treated with bCG for 6 weeks and only one person temporarily raised their testosterone levels to normal