Efficacy and safety of pulsatile GnRH pump therapy in male infants with congenital hypogonadotropic hypogonadism.

IF 2.8 3区 医学 Q3 ENDOCRINOLOGY & METABOLISM Endocrine Connections Pub Date : 2025-03-25 Print Date: 2025-05-01 DOI:10.1530/EC-24-0655
Xi Wang, Zi Xia Zhang, Jiajia Chen, Dongxia Fu, Mengmeng Du, Yongxing Chen, Haiyan Wei
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Abstract

Introduction: To investigate the efficacy and safety of pulsatile gonadotropin-releasing hormone pump therapy in male infants with congenital hypogonadotropic hypogonadism.

Methods: Seven male infants with congenital hypogonadotropic hypogonadism who were treated in a single center between 2019 and 2022 were included. Each patient was treated by subcutaneous infusion of 5 μg/90 min gonadorelin via a pulsatile gonadotropin-releasing hormone pump for 3-5 months. Data including age, drug dose, penile length, testicular volume and cryptorchidism were collected. Serum testosterone, follicle-stimulating hormone, luteinizing hormone, anti-Müllerian hormone, inhibin B concentration and liver and kidney functions were also measured. Data were analyzed using paired t-test and rank-sum test.

Results: Penile length increased from 1.27 ± 0.71 cm before treatment to 2.43 ± 0.90 cm after treatment, and testicular volume increased from 0.08 (0.04, 0.14) mL (left) and 0.08 (0.06, 0.12) mL (right) to 0.20 (0.16, 0.20) mL (left) and 0.22 (0.18, 0.30) mL (right). Inhibin B and anti-Müllerian hormone increased from 53.86 ± 11.54 pg/mL and 34.25 ± 12.48 ng/mL to 218.46 ± 32.34 pg/mL and 72.84 ± 9.24 ng/mL after treatment, respectively. At the end of the treatment, the testes of some children descended (6/13, 46.1%). During follow-up, the testes of two patients re-ascended to the inguinal region. No obvious adverse reactions were found in all patients before and after treatment.

Conclusion: Pulsatile gonadotropin-releasing hormone pump therapy can increase penile length, testicular volume, anti-Müllerian hormone levels and serum inhibin B levels in infants with congenital hypogonadotropic hypogonadism without adverse effects. However, its long-term efficacy and safety require further follow-up.

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搏动式GnRH泵治疗先天性促性腺功能减退症男婴的疗效和安全性。
目的:探讨搏动性促性腺激素释放激素泵治疗先天性促性腺功能减退症男婴的疗效和安全性。方法:选取2019 - 2022年间在同一中心接受治疗的7例先天性促性腺功能减退症男婴。患者采用脉动式促性腺激素释放激素泵皮下滴注促性腺激素5 μg/90 min,疗程3 ~ 5个月。收集年龄、药物剂量、阴茎长度、睾丸体积、隐睾等资料。测定血清睾酮、促卵泡激素、黄体生成素、抗勒氏激素、抑制素B浓度、肝肾功能。数据分析采用配对t检验和秩和检验。结果:阴茎长度由治疗前的1.27±0.71 cm增加到治疗后的2.43±0.90 cm,睾丸体积由0.08(0.04,0.14)mL(左)和0.08(0.06,0.12)mL(右)增加到0.20(0.16,0.20)mL(左)和0.22(0.18,0.30)mL(右)。抑制素B和抗勒根激素分别从53.86±11.54 pg/mL和34.25±12.48 ng/mL增加到218.46±32.34 pg/mL和72.84±9.24 ng/mL。治疗结束时,部分患儿睾丸下降(6/13,46.1%)。随访中,2例患者睾丸再次上升至腹股沟区。所有患者治疗前后均未见明显不良反应。结论:脉动式促性腺激素释放激素泵治疗可增加先天性促性腺功能减退症婴儿的阴茎长度、睾丸体积、抗勒氏激素水平及血清抑制素B水平,且无不良反应。但其长期疗效和安全性有待进一步随访。
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来源期刊
Endocrine Connections
Endocrine Connections Medicine-Internal Medicine
CiteScore
5.00
自引率
3.40%
发文量
361
审稿时长
6 weeks
期刊介绍: Endocrine Connections publishes original quality research and reviews in all areas of endocrinology, including papers that deal with non-classical tissues as source or targets of hormones and endocrine papers that have relevance to endocrine-related and intersecting disciplines and the wider biomedical community.
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