Guo Song, Zhang Jun, Li Yanhong, Ma Huamei, Chen Qiuli, Chen Hongshan, Du Minlian
Department of Pediatrics, The First Affiliated Hospital of Sun Yat-sen University, The 2nd Zhongshan Road, Number 58, Guangzhou city, Guangdong Province, China.
BMC Endocr Disord. 2019 Jul 11;19(1):72. doi: 10.1186/s12902-019-0403-2.
Further knowledge about the pubertal development mode of girls with Turner syndrome (TS) who have undergone hormone replacement therapy (HRT) is beneficial to the proposal of an optimal HRT regimen. This study examined the pubertal development mode of girls with TS who underwent HRT and evaluated the characteristics of optimal sex induction therapy in girls with TS.
We conducted a retrospective, longitudinal study over the past two decades at The First Affiliated Hospital, Sun Yat-sen University.
Seventy-one patients with TS and two groups of normal Chinese girls.
The total investigation time was 3.00 (2.00, 4.66) years. The interval of each stage was significantly longer (P < 0.001) in the girls with TS than that in the normal Chinese girls, except for B2-3 (P = 0.011). The uterine volumes of the girls with TS in stages B2 and 3 were greater than those of the control group (P = 0.046), whereas the uterine volume of the control group was inversely greater than that of the TS group among those who reached stages B4 and 5 (P = 0.034). During HRT, the uterine volume grew significantly from all previous stages except for breast stage 5 (B: Z = - 2.031; P = 0.042; B: Z = - 2.273; P = 0.023; B: Z = - 1.368; P = 0.171). The paired data of 27 girls with TS showed that the uterine volume (17.93 ± 9.31 ml vs. 13.75 ± 6.67 ml) and width (2.54 ± 0.66 cm vs. 2.22 ± 0.36 cm) increased significantly during artificial cycles compared with before artificial cycles (t = - 2.79 and - 2.51, P = 0.01 and 0.018).
HRT led to normal breast development in girls with TS; half of the girls with TS in our study reached Tanner stage B5, although the uterus ultimately developed suboptimally. The girls' breasts and uteruses grew quickly at the beginning of HRT (stages B2-4). An optimal HRT regimen for girls with TS may specifically focus on Tanner stages B2-4 and artificial cycles.
进一步了解接受激素替代疗法（HRT）的特纳综合征（TS）女孩的青春期发育模式，有助于提出最佳的HRT方案。本研究调查了接受HRT的TS女孩的青春期发育模式，并评估了TS女孩最佳性征诱导治疗的特点。
我们在中山大学附属第一医院进行了一项为期二十年的回顾性纵向研究。
71例TS患者以及两组正常中国女孩。
总调查时间为3.00（2.00，4.66）年。除B2-3期外（P = 0.011），TS女孩各阶段的间隔时间显著长于正常中国女孩（P < 0.001）。B2期和3期TS女孩的子宫体积大于对照组（P = 0.046），而在达到B4期和5期的女孩中，对照组的子宫体积反而大于TS组（P = 0.034）。在HRT期间，除乳腺5期外，子宫体积在所有先前阶段均显著增长（B：Z = - 2.031；P = 0.042；B：Z = - 2.273；P = 0.023；B：Z = - 1.368；P = 0.171）。27例TS女孩的配对数据显示，与人工周期前相比，人工周期期间子宫体积（17.93 ± 9.31 ml对13.75 ± 6.67 ml）和宽度（2.54 ± 0.66 cm对2.22 ± 0.36 cm）显著增加（t = - 2.79和 - 2.51，P = 0.01和0.018）。
HRT使TS女孩乳腺发育正常；本研究中一半的TS女孩达到坦纳B5期，尽管子宫最终发育欠佳。女孩的乳腺和子宫在HRT开始时（B2 - 4期）生长迅速。针对TS女孩的最佳HRT方案可能应特别关注坦纳B2 - 4期和人工周期。
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