Kirk J M, Savage M O, Grant D B, Bouloux P M, Besser G M
Department of Endocrinology, St Bartholomew's Hospital, London, UK.
Clin Endocrinol (Oxf). 1994 Jul;41(1):57-63. doi: 10.1111/j.1365-2265.1994.tb03785.x.
This study was designed to determine the response to therapy using human chorionic gonadotrophin (hCG) and human menopausal gonadotrophin (hMG) in males with idiopathic isolated hypogonadotrophic hypogonadism (IHH), and to compare the responses in patients presenting with and without cryptorchidism.
Analysis of male patients with IHH treated with hCG or combined hCG/hMG for a minimum of 6 months at St Bartholomew's Hospital. Clinical and endocrine assessment was performed in all patients prior to commencing therapy.
A total of 26 males with IHH have been treated with exogenous gonadotrophins. Thirteen patients (Group 1) had cryptorchidism (unilateral in 7, bilateral in 6) at presentation, and 13 (Group 2) did not.
All patients had basal serum testosterone, LH and FSH determinations. An i.v. GnRH test was performed in 25 patients and an i.m. hCG stimulation test in 19. Testicular volume and serum testosterone were measured during both hCG and combined hCG/hMG therapy. Seminal analysis was performed at the start and monthly during hCG/hMG therapy.
Eighty-five per cent of the 13 patients in Group 1 had an olfactory defect (Kallmann's syndrome), compared with 23% of Group 2. Both groups of patients showed a subnormal response to initial i.v. GnRH and i.m. hCG testing. During hCG therapy only three patients in Group 1 and six in Group 2 achieved normal adult testosterone levels. The non-cryptorchid group achieved a higher mean testicular volume on hCG therapy than the cryptorchid group (mean (SD); 4.7 (1.8) ml vs 3.0 (1.6) ml (P < 0.02)), and for all patients there was a correlation between initial and maximal testicular volume (R = 0.69, P = 0.001). Four patients in Group 1 and five patients in Group 2 were treated with combined hCG/hMG for 6-15 months to induce fertility; only one patient in Group 1 achieved spermatogenesis, compared to all patients in Group 2 (leading to three pregnancies).
These data indicate that patients with idiopathic hypogonadotrophic hypogonadism (IHH) have a poor response to hCG therapy in terms of testicular growth and normalization of serum testosterone. Final testicular volume is dependent on initial testicular size. In addition, patients with IHH associated with cryptorchidism have a poor fertility potential to combined hCG/hMG therapy.
本研究旨在确定特发性孤立性低促性腺激素性性腺功能减退症（IHH）男性患者对人绒毛膜促性腺激素（hCG）和人绝经期促性腺激素（hMG）治疗的反应，并比较伴有和不伴有隐睾症患者的反应。
对在圣巴塞洛缪医院接受hCG或联合hCG/hMG治疗至少6个月的IHH男性患者进行分析。在开始治疗前，对所有患者进行临床和内分泌评估。
共有26例IHH男性患者接受了外源性促性腺激素治疗。13例患者（第1组）就诊时患有隐睾症（单侧7例，双侧6例），13例（第2组）没有隐睾症。
所有患者均测定基础血清睾酮、促黄体生成素（LH）和促卵泡生成素（FSH）。25例患者进行了静脉注射促性腺激素释放激素（GnRH）试验，19例进行了肌肉注射hCG刺激试验。在hCG和联合hCG/hMG治疗期间测量睾丸体积和血清睾酮。在hCG/hMG治疗开始时及每月进行一次精液分析。
第1组13例患者中有85%存在嗅觉缺陷（卡尔曼综合征），而第2组为23%。两组患者对初始静脉注射GnRH和肌肉注射hCG试验的反应均低于正常水平。在hCG治疗期间，第1组只有3例患者，第2组有6例患者达到正常成人睾酮水平。非隐睾组在hCG治疗时的平均睾丸体积高于隐睾组（均值（标准差）；4.7（1.8）ml对3.0（1.6）ml（P<0.02）），并且对于所有患者，初始睾丸体积与最大睾丸体积之间存在相关性（R=0.69，P=0.001）。第1组4例患者和第2组5例患者接受联合hCG/hMG治疗6 - 15个月以诱导生育；第1组只有1例患者实现精子发生，而第2组所有患者均实现（导致3次妊娠）。
这些数据表明，特发性低促性腺激素性性腺功能减退症（IHH）患者在睾丸生长和血清睾酮正常化方面对hCG治疗反应不佳。最终睾丸体积取决于初始睾丸大小。此外，伴有隐睾症的IHH患者对联合hCG/hMG治疗的生育潜力较差。
