Abstract:

Objective　To research an appropriate estrogen therapy for in the pubertal development in Turner syndrome （TS） achieved by estradiol valerate. Methods　In 57 TS girls of no spontaneous puberty or puberty arrest，we retrospectively studied pubertal stage and uterine dimension during the estrogen replacement therapy. Data from patient records was collected，described the pubertal developing procedure，and compared in groups which grouped by estrogen dosage to detect an appropriate dosage and that can lead a better breast and uterine development. Results　The median age at start of puberty induction was 15.00 years，with a range of 11.5-21.0 years. （1） Breast development：Breast development to Tanner stage B2 was achieved in 0.29（0.25-0.33） years，stage B3 in 0.75（0.46，1.08） years，stage B4 in 2.20（0.92，3.08） years and B5 in 3.67（1.71，4.44） years. （2）Uterine development：The uterine volume and length in TS girls before treatment was 0.51（0.14，0.86）ml and 1.89（1.23，2.18） cm. We grouped the patients of Tanner stage B2 in groups of estrogen dosage ≤0.5 mg/d and＞0.5 mg/d and the uterine dimension and weight showed no difference. When the patients were grouped as groups of dosage＜1.0 mg/d and group of dosage≥1.0 mg/d in stage B3，the uterine indexes in lower dosage group were less than group with larger dosage. When they were grouped as groups of dosage＜1.5 mg/d and ≥1.5 mg/d，the uterine volume 6.96（3.15-11.00） ml in lower dosage group was smaller than that in group with larger dosage. Conclusion　During estrogen treatment in TS girls，normal breast development can be achieved. In a clinical setting，the uterine volume and length under pubertal induction developed properly with the breast stages progressing. when the breast developed to stage B2，the uterine development was more dependable on estrogen. We recommend a low daily beginning estrogen dosage until stage B2，which can be increased gradually after B2 to speed up the uterine development.

Key words: Turner syndrome, estrogen replacement treatment, breast development, uterus

摘要：

目的　观察以雌激素替代诱导Turner综合征患儿乳房和子宫发育进程的规律，探究获得模拟正常青春发育进程的疗效与雌激素剂量和疗程的关系。方法　2005年1月至2015年12月期间，对就诊于中山大学附属第一医院儿科57例无青春发育或发育停滞的Turner综合征患儿，按国际指南方案治疗，以雌激素小剂量开始，逐步递增。回顾性分析雌激素剂量调节方案与乳房Tanner分期和子宫发育进程的关系。结果　57例Turner综合征患儿开始雌激素替代治疗年龄为15.00（14.25，16.87）岁，追踪时间2.07（0.62，3.06）年。（1）乳房发育：追踪年限内达到B2、B3、B4和B5期所需时间分别为0.29 （0.25，0.33），0.75 （0.46，1.08），2.20 （0.92，3.08）和3.67 （1.71，4.44）年。（2）子宫发育：雌激素替代治疗前，Turner综合征患儿子宫容积0.51（0.14，0.86） mL，长径1.89（1.23，2.18） cm。替代后乳房达B2期时，雌激素剂量≤0.5 mg/d组与＞0.5 mg/d组的子宫容积和长径差异无统计学意义。B3期时，雌激素≥1.0 mg/d组的子宫参数均大于雌激素＜1.0 mg/d组。B4期时，雌激素≥1.5 mg/d组的子宫容积较＜1.5 mg/d组子宫容积大。结论　Turner综合征患儿接受雌激素替代治疗后，乳房发育进程可接近正常。子宫发育随乳房Tanner分期进展。在乳房发育达B2期后，子宫发育进程对雌激素依赖性增加。雌激素替代治疗时，起始可予小剂量，达B2期后可适当加大剂量并递增，以加速子宫发育。

关键词: Turner综合征, 雌激素替代疗法, 乳房发育, 子宫