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[先天性孤立性促性腺激素缺乏性性腺功能减退男孩的青春期诱导]

[Puberty induction in boys with congenital isolated hypogonadotropic hypogonadism].

作者信息

Kokoreva K D, Chugunov I S, Kareva M A, Bezlepkina O B

机构信息

Endocrinology Research Centre.

出版信息

Probl Endokrinol (Mosk). 2023 Feb 25;69(1):59-67. doi: 10.14341/probl13141.

Abstract

BACKGROUND

Gonadotropin therapy in boys with congenital isolated hypogonadotropic hypogonadism helps to increase testes volume and induce spermatogenesis in comparison with testosterone therapy. However, difficulties with dose titration, partial therapy success, absence of generally accepted regimen protocols don't allow to use this therapy in order to induce puberty in adolescents with Kallmann syndrome or normosmic hypogonadotropic hypogonadism.

AIM

To assess the effectiveness of combination hormonal replacement therapy via human chorionic gonadotropin and recombinant follicle stimulation hormone in adolescents with congenital isolated normosmic hypogonadotropic hypogonadism and with Kallmann syndromeMATERIALS AND METHODS: This is an open single-center prospective non-controlled study. Boys with hypogonadotropic hypogonadism were receiving hormonal replacement therapy for 12 months. Initial dose of human chorionic gonadotropin was 500 IU per week. Initial dose of recombinant follicle stimulation hormone was 37.5 IU per week. Doses were doubled in 6 months. Antropometric data, Tanner stage, testes volumes, inhibin B and anti-Mullerian hormone (AMH) levels were evaluated in all the patients before the treatment, after 6 and 12 months of the therapy.

RESULTS

8 boys with hypogonadotropic hypogonadism were included into the study. Median age before therapy initiation was 15.7 years [15.33; 16.41]. In 12 months after the therapy initiation puberty development, testosterone increase from 0.44 [0.34;0.62] to 4.39 [0.88;10.51] nmol/l (p=0.012), AMH decrease from 35.70 [18.00;59.00] to 14.41 [11.60;16.65] ng/ml were noted in all the patients (p=0.017). Testes volumes increase and inhibin B level increase were not statistically significant.

CONCLUSION

Gonadotropin therapy is effective in order to puberty initiation in adolescents with congenital hypogonadotropic hypogonadism. In helps to achieve not only androgenization, but also to Sertoli cells maturation.

摘要

背景

与睾酮治疗相比,先天性孤立性低促性腺激素性性腺功能减退男孩接受促性腺激素治疗有助于增加睾丸体积并诱导精子发生。然而,剂量滴定困难、部分治疗成功、缺乏普遍接受的治疗方案,使得无法使用这种疗法来诱导卡尔曼综合征或嗅觉正常的低促性腺激素性性腺功能减退青少年进入青春期。

目的

评估人绒毛膜促性腺激素和重组卵泡刺激素联合激素替代疗法对先天性孤立性嗅觉正常的低促性腺激素性性腺功能减退和卡尔曼综合征青少年的有效性。

材料与方法

这是一项开放的单中心前瞻性非对照研究。低促性腺激素性性腺功能减退男孩接受激素替代治疗12个月。人绒毛膜促性腺激素初始剂量为每周500国际单位。重组卵泡刺激素初始剂量为每周37.5国际单位。6个月后剂量加倍。在治疗前、治疗6个月和12个月后评估所有患者的人体测量数据、 Tanner分期、睾丸体积、抑制素B和抗苗勒管激素(AMH)水平。

结果

8名低促性腺激素性性腺功能减退男孩纳入研究。治疗开始前的中位年龄为15.7岁[15.33;16.41]。治疗开始12个月后,所有患者均出现青春期发育,睾酮从0.44[0.34;0.62]升至4.39[0.88;10.51]nmol/l(p=0.012),AMH从35.70[18.00;59.00]降至14.41[11.60;16.65]ng/ml(p=0.017)。睾丸体积增加和抑制素B水平升高无统计学意义。

结论

促性腺激素治疗对先天性低促性腺激素性性腺功能减退青少年启动青春期有效。它不仅有助于实现雄激素化,还有助于支持细胞成熟。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d5e/9978876/94be00b58bb4/problendo-69-13141-g001.jpg

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