中国实用儿科杂志

中国实用儿科杂志

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Turner综合征的卵巢功能评估和激素替代治疗

  

  1. 重庆医科大学附属儿童医院内分泌遗传代谢科  国家儿童健康与疾病临床医学研究中心  儿童发育疾病研究教育部重点实验室  儿科学重庆市重点实验室，重庆  400014
  • 出版日期:2021-08-06 发布日期:2021-09-09

Ovarian function assessment and hormone replacement therapy in Turner syndrome

  1. Department of Endocrinology，Genetics and Metabolism，Children’s Hospital of Chongqing Medical University；National Clinical Research Center for Child Health and Disorders；Ministry of Education Key Laboratory of Child Development and Disorders；Chongqing Key Laboratory of Pediatrics，Chongqing  400014，China
  • Online:2021-08-06 Published:2021-09-09

摘要： 特纳综合征（TS）通常有高促性腺激素性性腺功能减退症、 原发性或继发性闭经。因此， 大多数TS患者需要激素替代疗法（HRT）来诱导青春期， 维持第二性征发育，使子宫正常生长， 并获得峰值骨量。激素替代的目的是模拟正常的身心发育。诱导青春期发育的最佳激素替代治疗方案仍在不断的优化和改进。治疗应从11～12岁开始， 在2～3年内每6个月增加一次剂量。低剂量的雌激素启动青春期对于保护生长潜力至关重要。该文重点介绍了在TS年轻患者中的性激素替代疗法， 期望为临床医生提供实际帮助。

关键词: 特纳综合征, 激素替代治疗, 卵巢功能评估, 雌激素, 孕激素

Abstract: Turner syndrome is usually accompanied by hypergonadotropic hypogonadism and primary or secondary amenorrhea. Most TS individuals will therefore need hormonal replacement therapy（HRT） for induction of puberty and later for maintaining secondary sex characteristics，attaining peak bone mass，normalizing uterine growth. The goals of hormone replacement are to mimic the normal physical and social development. The optimal hormone replacement treatment regimen to induce pubertal development is still being determined and improved. Treatment should begin at 11-12 years of age，with dose increasing every 6 months over a 2-3-year period. Initiation of puberty with low doses of estrogen is crucial to preserve growth potential. This paper discusses the sex steroid replacement therapy in younger patients with Turner syndrome，aiming to provide practical help for clinicians.

Key words: Turner syndrome, hormonal replacement therapy, ovarian function assessment, estrogen, progestogen