Bannink E M N, van Sassen C, van Buuren S, de Jong F H, Lequin M, Mulder P G H, de Muinck Keizer-Schrama S M P F
Department of Paediatrics, Division of Endocrinology, Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands.
Clin Endocrinol (Oxf). 2009 Feb;70(2):265-73. doi: 10.1111/j.1365-2265.2008.03446.x.
Besides short stature, gonadal dysgenesis leading to a lack of oestrogen is one of the main characteristics of Turner syndrome (TS). In most TS girls, puberty is induced with exogenous oestrogens.
To describe the pubertal development and uterine dimensions achieved by low-dose 17beta-oestradiol (17beta-E2) orally started at an appropriate age. Additionally, to determine whether serum hormone levels aid evaluation of pubertal progression.
In 56 TS girls, we prospectively studied pubertal stage, serum E2, LH, FSH, SHBG and oestrone (E1), starting oestrogen treatment with a low-dose 17beta-E2 (5 microg/kg/day) during GH treatment at mean (SD) age 12.7 (0.7) years. Hormone levels were measured at start, 3 months after start and after increasing 17beta-E2 dosage. Uterine dimensions were measured in 39 TS women at age 19.9 (2.2) years.
Although breast and pubic hair development were similar to that in normal Dutch girls up to Tanner stage B5 and P5, respectively, breast development was 2 years later. Before oestrogen therapy, E2 levels were comparable to those in prepubertal girls. With a 17beta-E2 dose of 5 microg/kg/day, these levels increased significantly, becoming comparable to normal late pubertal or adult concentrations, whereas SHBG levels were unchanged. At the adult 17beta-E2 dose, SHBG had increased significantly. Uterus shape was juvenile in four (10.2%), cylindrical in four and mature-adult shaped in 31 (79.5%) of TS patients.
During GH treatment in TS girls, normal breast development up to B5 can be mimicked, with just a 2-year delay. In a clinical setting, serum hormone levels provide no additional information for evaluating pubertal progression. After age-appropriate pubertal induction, uterine dimensions in women aged nearly 20 years were subnormal. It remains unclear whether this was related to E2 dosage, timing or duration, or factors related to TS.
除身材矮小外，性腺发育不全导致雌激素缺乏是特纳综合征（TS）的主要特征之一。在大多数TS女孩中，青春期通过外源性雌激素诱导。
描述在适当年龄开始口服低剂量17β-雌二醇（17β-E2）所实现的青春期发育和子宫大小。此外，确定血清激素水平是否有助于评估青春期进展。
在56名TS女孩中，我们前瞻性地研究了青春期阶段、血清E2、LH、FSH、SHBG和雌酮（E1），在平均（标准差）年龄12.7（0.7）岁的生长激素（GH）治疗期间开始用低剂量17β-E2（5微克/千克/天）进行雌激素治疗。在开始时、开始后3个月以及增加17β-E2剂量后测量激素水平。在39名年龄为19.9（2.2）岁的TS女性中测量子宫大小。
尽管乳房和阴毛发育分别与正常荷兰女孩在坦纳分期B5和P5之前相似，但乳房发育延迟了2年。在雌激素治疗前，E2水平与青春期前女孩相当。使用5微克/千克/天的17β-E2剂量时，这些水平显著升高，与正常青春期后期或成人浓度相当，而SHBG水平未改变。在成人17β-E2剂量时，SHBG显著升高。TS患者中，4例（10.2%）子宫形状为幼稚型，4例为圆柱形，31例（79.5%）为成熟成人型。
在TS女孩的GH治疗期间，可以模拟出直至B5的正常乳房发育，只是延迟2年。在临床环境中，血清激素水平对于评估青春期进展没有提供额外信息。在适当年龄进行青春期诱导后，近20岁女性的子宫大小低于正常。尚不清楚这是否与E2剂量、时机或持续时间有关，还是与TS相关因素有关。
