Investigational Treatment of Congenital Hypogonadotropic Hypogonadism in Infants.

IF 2 4区 医学 Q3 ENDOCRINOLOGY & METABOLISM
Xiaoya Ren, Yi Wang, Ying Liu, Chunxiu Gong
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Abstract

The aim of the study was to investigate whether the administration of gonadotropins to mimic the physiological development of infants with congenital hypogonadotropic hypogonadism (CHH) after birth can facilitate testicular descent, penile growth, and ultimately preserve fertility. This study included eight infants with CHH who received a gonadotropin-releasing hormone (GnRH) pump or human chorionic gonadotropin (HCG) combined with human menopausal gonadotropin (HMG) therapy at Beijing Children's Hospital from August 2018 to March 2023. The age of the infants ranged from 6 months to 2 years. 2. For literature review, a search was conducted in the PubMed database using the keywords "congenital hypogonadotropic hypogonadism," "infants," and "mini-puberty" up until June 2023. After 1-3 months of treatment, significant increases were observed in PL and TV. The testes descended from the inguinal region to the scrotum. Serum T and INH-B levels increased from being undetectable to 737.1±409.5 ng/dl and from 47.88±23.03 to 168.94±59.34 pg/ml, respectively. In a comparative literature review of 22 infants with CHH, the age at treatment initiation ranged from 0.5 to 7.9 months. Treatment involved various dosages and durations, ranging from 2 to 6 months of subcutaneous injections of LH and FSH. Both therapies successfully improved PL, TV, and testicular descent; reduced the need for surgery; and were safe. This is the first report of the use of a GnRH pump for the treatment of infant CHH.

婴儿先天性性腺功能减退症的研究性治疗。
该研究的目的是探讨在患有先天性性腺功能减退症(CHH)的婴儿出生后给予促性腺激素以模拟其生理发育,是否能促进睾丸下降、阴茎生长并最终保留生育能力。本研究纳入了2018年8月至2023年3月期间在北京儿童医院接受促性腺激素释放激素(GnRH)泵或人绒毛膜促性腺激素(HCG)联合人绝经期促性腺激素(HMG)治疗的8名CHH患儿。婴儿年龄从6个月到2岁不等。2.文献综述方面,在PubMed数据库中以 "先天性性腺功能减退症"、"婴儿 "和 "小青春期 "为关键词进行了检索,直至2023年6月。治疗 1-3 个月后,观察到 PL 和 TV 显著增加。睾丸从腹股沟区下降到阴囊。血清T和INH-B水平分别从检测不到增加到737.1±409.5 ng/dl和从47.88±23.03增加到168.94±59.34 pg/ml。在对 22 例 CHH 婴儿的文献比较中,开始治疗的年龄从 0.5 个月到 7.9 个月不等。治疗涉及不同剂量和持续时间,皮下注射 LH 和 FSH 的时间从 2 个月到 6 个月不等。两种疗法都成功地改善了PL、TV和睾丸下降,减少了手术需求,而且安全可靠。这是第一份使用 GnRH 泵治疗婴儿 CHH 的报告。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Hormone and Metabolic Research
Hormone and Metabolic Research 医学-内分泌学与代谢
CiteScore
3.80
自引率
0.00%
发文量
125
审稿时长
3-8 weeks
期刊介绍: Covering the fields of endocrinology and metabolism from both, a clinical and basic science perspective, this well regarded journal publishes original articles, and short communications on cutting edge topics. Speedy publication time is given high priority, ensuring that endocrinologists worldwide get timely, fast-breaking information as it happens. Hormone and Metabolic Research presents reviews, original papers, and short communications, and includes a section on Innovative Methods. With a preference for experimental over observational studies, this journal disseminates new and reliable experimental data from across the field of endocrinology and metabolism to researchers, scientists and doctors world-wide.
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