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图纳综合征女孩在青春期诱导期间的生长:回顾性队列分析

Joshua Nicholls^1,2, Mark Harris^2,3, Ian Hughes⁴

¹Department of Paediatrics, Queensland Children's Hospital, South Brisbane 4101, Queensland, Australia.

The Journal of clinical endocrinology and metabolism

|February 9, 2024

概括

为特纳综合征 (TS) 患者优化青春期诱导是关键. 个性化的时间和精细的雌激素治疗对于最大限度地提高成人身高 (AH) 和改善生长结果至关重要.

科学领域:

儿科内分泌学儿科内分泌学
遗传学遗传学是一个
增长和发展增长和发展

背景情况:

特纳综合征 (TS) 经常导致矮身和卵巢缺陷.
在TS患者中,诱导青春期以最大限度地提高成年人身高 (AH) 的最佳策略仍未确定.

研究的目的:

在接受青春期诱导的特纳综合征患者中确定影响生长和AH的因素.
评估诸如诱导时的年龄,骨龄和生长激素治疗对结果的影响.

主要方法:

在专科诊所对107名TS患者 (2015-2021) 的回顾性队列研究.
对51名接受雌醇治疗以诱导青春期的患者的分析.
对AH和身高标准偏差得分 (ΔHeightSDS) 的预测因子的评估,包括父母的中等身高 (MPH),骨龄延迟和生长激素 (GH) 剂量.

主要成果:

成年人身高 (AH) 受到父母身高中等 (MPH) 和骨年龄延迟的显著影响.
高度标准偏差得分变化 (ΔHeightSDS) 根据GH剂量,骨年龄延迟和青春期诱导时的年龄进行预测.
骨年龄延迟和诱导年龄之间的相互作用表明,较早的诱导与较少的骨年龄延迟与较低的ΔHeightSDS相关.

结论:

在TS患者中,青春期诱导时间需要个性化的方法.
目前的雌激素补充方案可能需要调整,以支持生理性青春期诱导,而不会影响最终的身高.

关键词:

图纳综合征是特纳综合征的一种症状.增长激素增长激素的使用方法这是青春期诱导.

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