迷你青春期,生理和失调:后果,以及治疗替代的潜力.
Julia Rohayem1,2, Emma C Alexander3, Sabine Heger4
1Department of Pediatric Endocrinology and Diabetology, Children's Hospital of Eastern Switzerland, 9006 St. Gallen, Switzerland.
Endocrine reviews
|March 4, 2024
概括
遗传性性性性性 (CHH) 扰乱了男性婴儿的关键"迷你青春期"阶段,影响了未来的生育能力. 早期诊断和淋巴激素治疗可以改善生殖结果和身体发育.
科学领域:
- 生殖内分泌学 生殖内分泌学
- 儿科内分泌学 儿科内分泌学
- 遗传学 是一个遗传学.
背景情况:
- 下垂体-垂体-腺 (HPG) 轴表现出三个不同的生理波:胎儿,迷你青春期和青春期.
- 先天性性性性性 (CHH) 是一种遗传性疾病,其特征是缺少性释激素 (GnRH) 的分泌或作用,在所有三个HPG轴波中都不存在.
- 在男性中,严重的CHH可能会导致微阴茎,密码化和丸不成熟,这是由于缺少胎儿和迷你青春期的HPG轴激活,这会影响未来的生育能力.
研究的目的:
- 审查迷你青春期的生理学和中枢HPG轴障碍的后果.
- 概述一种诊断方法,用于在婴儿中早期发现CHH.
- 检查当前的治疗策略,以CHH替代男婴的迷你青春期.
主要方法:
- 对生理学迷你青春期和中央HPG轴障碍的审查.
- 对CHH的诊断方法的讨论.
- 对CHH当前治疗选择的分析,包括淋巴激素替代疗法.
主要成果:
- 胎儿HPG轴激活的缺失发生在约50%的男性新生儿中,这些新生儿患有小阴茎和/或密码性阴茎.
- 缺乏迷你青春期会加剧丸不成熟,以低的塞尔托利细胞数量为特征,对未来的生育能力产生不利影响.
- 小案例系列表明,模仿迷你青春期的淋巴激素替代可以改善丸下降,使丸和阴茎大小正常化,并解决生殖和非生殖方面的影响.
结论:
- 在婴儿中,CHH诊断经常被错过,没有建立的治疗共识.
- 早期识别和干预,包括以模仿小青春期的方式替代性腺激素,有望改善受影响男性的生殖结果.
- 治疗性替代疗法治疗失调的小青春期可能会提供超越身体发育的好处,解决更广泛的生殖健康问题.
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