特发性低促性腺激素性性腺功能减退症2例临床观察

Clinical observation of 2 cases of idiopathic hypogonadotropin hypogonadism

目的报道兰州大学第二医院内分泌与代谢科2017年—2018年收治的2例主因第二性征发育不全就诊的特发性低促性腺激素性性腺功能减退症(IHH)青年患者,2例患者就诊年龄、身高及青春发育水平相似,分别选择了促性腺激素释放激素(GnRH)泵、人绒毛膜促性腺激素(HCG)治疗,通过观察2例患者治疗后的激素水平变化、第二性征发育情况评估两种治疗方案的效果。方法病例1选择GnRH泵(戈那瑞林)10μg/90min脉冲式输注治疗;病例2选择注射用绒促性素2 000U肌内注射3次/周治疗。结果对2例患者治疗效果观察1~2年,发现其治疗3~6个月后青春发育明显,第二性征发育水平接近成人,可进行正常性生活,卵泡刺激素(FSH)、黄体生成素(LH)及睾酮(T)水平逐步升高。结论通过对2例患者的疗效观察,在GnRH泵或HCG长期、规范治疗的前提下,IHH可以有效治疗且治疗效果良好,且GnRH泵脉冲式治疗效果优于单纯HCG治疗。

Objective 2 young patients with idiopathic hypogonadotropin hypogonadism(IHH) who were treated in the Department of Endocrinology and Metabolism of Lanzhou University Second Hospital from 2017 to 2018 were reported. The age, height and youth development level of the two patients were similar. Gonadotropin-releasing hormone(GnRH) pump and human chorionic gonadotropin(HCG) were selected for treatment respectively, and the effects of the two treatments were evaluated by observing the changes of hormone levels and the development of secondary sexual characteristics in 2 patients after treatment. Methods Case 1 was treated with GnRH pump(gonarelin) pulse infusion at 10μg/90 min. Case 2 was treated by intramuscular injection of 2 000 U chorionic tropin 3 times per week. Results The treatment effect of 2 patients was observed for 1-2 years. It was found that the youth development was obvious after 3-6 months of treatment, the development level of secondary sexual characteristics was close to that of adults, and the normal sexual life could be carried out. The levels of FSH, LH and T gradually increased. Conclusions Through the observation of the efficacy of 2 patients, IHH can be effectively treated and the therapeutic effect is good under the premise of long-term and standardized treatment with GnRH pump or HCG, and the effect of GnRH pump pulse therapy is better than that of HCG alone.