生长激素和低剂量雌激素治疗特纳综合征女孩的最终身高结局

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摘要

我们报告了28例Turner综合征(TS)患者在身高生长激素治疗和晚期雌激素治疗期间的最终身高数据经染色体分析证实。参与者被分为3组,A组:11例15-18岁的患者,B组:10例10 -15岁的患者,C组:7例10岁以下的患者。与使用生长激素的时间和呈现时间相比,B组的最终身高平均值(147.5±6.5 cm)显著高于B组,且使用长时间高剂量生长激素的B组效果最好。对经核型分析证实的女性TS患者进行记录检查;15例患者具有典型的特纳核型,13例患者具有表明TS变异之一的核型。16个变异个体中有4个缺乏与特纳综合征相关的临床红斑;另外9例有一个或多个与TS相关的临床常见的柱头。2例具有复杂的马赛克核型的患者还具有Y染色体。经组主动脉缩窄3例,TS变异型1例,原发性甲状腺功能减退5例,行左旋甲状腺素治疗。两名PTS有甲状腺功能亢进。在特纳综合征(TS)中,生长激素(GH)治疗促进生长和成人身高。用弱雄激素奥雄龙(Ox)补充生长激素的利弊比尚未确定。8岁开始使用生长激素(1.33 mg/kg/d), 12岁开始使用雌激素。系统评价成人身高(成人身高减去预测成人身高)的增长和安全系数。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Final Height Outcome in Girls with Turner Syndrome Treated with Growth Hormone and Low Dose Estrogen
We reported final height data of collected 28 cases with Turner Syndrome (TS) were confirmed with chromosomal analysis during height growth hormone therapy and late estrogen therapy. Participants were allocated into one of 3 Groups, Group A: 11 patients aged between 15-18 years-old, group B: 10 patients aged between 10 -15 years-old, group C: 7 patients younger than 10 years-old. In contrast to the duration of growth hormone & presentation, group B showed a significantly higher final height mean (147.5 ±6.5 cm) the best result well be obtained in group B treated with high doses of growth hormone for longer period. Records of female patients with TS confirmed by karyotype analysis were examined; 15 patients had the typical Turner karyotype and 13 patients had a karyotype indicative of one of the TS variations. Four of the sixteen individuals in variations lacked clinical stigmata associated with Turner Syndrome; the other nine had one or more of the usual clinical stigmata associated with TS. Two cases who had a complicated mosaic karyotype also possessed a Y chromosome. Three patients in group of classics had coarctation of the aorta and one patient in had variants of TS. 5 patients had primary hypothyroidism and received levothyroxine. two pts has hyperthyroidism. In Turner syndrome (TS), Growth Hormone (GH) treatment promotes growth and adult height. The benefit-risk ratio of supplementing GH with the weak androgen oxandrolone (Ox) is undefined. Growth hormone were administered (1.33 mg/kg/d) from the age of 8 years-old while estrogens were initiated at the age of 12 years-old. The increase in adult height (adult height minus predicted adult height) and safety factors were evaluated in a systematic manner.
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