特发性低促性腺激素性性腺功能减退症回顾性研究

A retrospective study of idiopathic hypogonadotropic hypogonadism

目的总结男性特发性低促性腺激素性性腺功能减退症(IHH)临床特点,探讨治疗方法及疗效。方法回顾性分析45例男性IHH患者的病史资料。结果 45例男性IHH患者中,无嗅觉丧失或障碍,即嗅觉正常的IHH(Norm-osmic IHH,n IHH)20例,伴有嗅觉异常或丧失的IHH,即卡尔曼综合征(Kallmann Syndrome,KS)25例。KS组和n IHH组患者黄体生成素(LH)、卵泡刺激素(FSH)、血清睾酮(T)性激素水平分别为(0.62±0.30)IU/L、(0.82±0.40)IU/L、(0.93±0.52)nmol/L和(0.64±0.28)IU/L、(0.68±0.43)IU/L、(0.82±0.57)nmol/L,差异无统计学意义(P>0.05);KS组和n IHH组患者隐睾发生率、微小阴茎、睾丸体积分别为32.0%、24.0%、(3.63±1.26)ml和30.0%、20.0%、(4.23±1.43)m L,差异无统计学意义(P>0.05)。所有患者均治疗6个月以上,疗程(11.2±4.2)月,治疗后体力改善,第二性征不同程度的发育。末次统计时患者睾丸体积、阴茎长度、阴毛Tanner分期均显著优于治疗前(P<0.01)。9例患者进行了精液分析,其中8例可见精子,但精子活力低于正常标准。结论 IHH患者主要表现为第二性征发育障碍,性激素水平低下。n IHH型和KS型临床特征无显著差异,HCG联合雄激素是治疗IHH的有效的方法。

Objective To analyze the clinical characteristics of idiopathic hypogonadotropic hypogonadism（IHH）, andto explore the treatment and effect. Methods A total of 45 cases of male IHH patients＇ medical history information wereretrospectively studied. Results In 45 cases of male patients with IHH, 20 cases were Normosmic IHH（n IHH type）, withoutthe sense of smell loss or impairment, and 25 cases were Kallmann Syndrome（KS type）, accompanied by abnormal smell orloss. The luteinizing hormone（LH）, follicle-stimulating hormone（FSH）, serum testosterone（T） hormone levels in KS group[（0.62±0.30）IU/L、（0.82±0.40）IU/L、（0.93±0.52）nmol/L] and n IHH group [（0.64±0.28）IU/L、（0.68±0.43）IU/L、（0.82±0.57）nmol/L], were with no statistically significant difference（P〉0.05）; The incidence of cryptorchidism, small penis, and testicularvolume were 32.0%、24.0%、（3.63±1.26）m L in KS group, and 30.0%、20.0%、（4.23±1.43）m L in n IHH group, there was nostatistically significant difference（P〉0.05）. All the patients were treated for more than 6 months, the average course oftreatment were（11.2± 4.2） months. After the treatment, the physical strength and secondary sex characteristic were developedin different levels. The testicular volume, penile length, and Tanner staging after treatment were significantly improved thanbefore treatment（P〈0.01）. Sperm analysis was carried out in 9 patients, sperm were found in 8 patients, but the sperm activitywere lower than that of the normal standard. Conclusion The mainly manifestation of IHH patients are secondary sexualcharacteristic developmental disorders and low sex hormone levels.There are no significant difference in clinical characteristicsbetween KS and n IHH. HCG combined with androgen is an effective method for the treatment of IHH.