延迟青春期的频率在男孩与杜申肌肉发育不良的当代管理的杜申肌肉发育不良
Sarah McCarrison1, Melissa Denker1, Jennifer Dunne2
1Royal Hospital for Children, Clinic of Paediatric Endocrinology, Glasgow, United Kingdom
Journal of clinical research in pediatric endocrinology
|June 25, 2024
概括
延迟青春期影响65%的杜申肌力发育不良 (DMD) 的男孩,特别是那些每天接受葡萄糖皮质激素治疗的人. 建议对这些患者进行定期的青春期监测.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 神经肌肉疾病 神经肌肉疾病
- 青少年健康 青少年健康
背景情况:
- 延迟的青春期是杜申肌力发育不良 (DMD) 的男孩的一个公认问题.
- 长期口服葡萄糖皮质激素治疗在DMD治疗中很常见,可能会影响青春期的发展.
研究的目的:
- 为了确定杜申肌力发育不良的男孩延迟青春期的频率.
- 调查葡萄糖皮质激素治疗与DMD中青春期计时之间的关联.
- 为了描述男孩的内分泌特征,青春期延迟和DMD.
主要方法:
- 对14岁以上的DMD男孩进行了回顾性研究 (2016-2022年).
- 根据丸体积和生殖器分期使用青春期名谱来评估延迟的青春期.
- 详细的内分泌检查,包括激素水平测量和刺激测试.
主要成果:
- 在37名患有DMD的男孩中,65%的男孩表现出延迟的青春期.
- 在每天接受葡萄糖皮质醇治疗的男孩中,82%的男孩显示出延迟青春期的证据.
- 内分泌检测显示功能性中央性阴性腺症,其特征是低素激素和水平.
结论:
- 晚期青春期在患有DMD的男孩中非常普遍,与每日使用葡萄糖皮质激素有很强的相关性.
- 功能性中枢性低子腺症似乎是潜在的内分泌问题.
- 对于患有杜申肌力发育不良的青少年男孩来说,常规的青春期监测是必不可少的.
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