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特纳综合征患者骨密度降低的相关因素:一项多中心前瞻性观察研究。

Factors associated with low bone mineral density in Turner syndrome: a multicenter prospective observational study.

机构信息

Division of Endocrinology and Metabolism, Tokyo Metropolitan Children's Medical Center, Fuchu 183-8561, Japan.

Clinical Research Support Center, Tokyo Metropolitan Children's Medical Center, Fuchu 183-8561, Japan.

出版信息

Endocr J. 2024 Jun 18;71(6):561-569. doi: 10.1507/endocrj.EJ23-0628. Epub 2024 Mar 22.

Abstract

Turner syndrome (TS) is associated with a high risk of fracture due to low bone mineral density (BMD). While hypogonadism is known to play a role in decreasing BMD, other factors have not been studied well. Focusing on diet, exercise, and bone metabolism markers, the present, multicentric, prospective, observational study aimed to identify factors contributing to decreased BMD in TS. In total, 48 patients with TS aged between 5 and 49 years comprising a pre-pubertal group (n = 9), a cyclical menstruation group (n = 6), and a hormone replacement therapy (HRT) group (n = 33) were enrolled. The cyclical menstruation group and the HRT group were referred to collectively as the post-pubertal group. The bone mineral apparent density (BMAD) Z-score was higher in the pre-pubertal group than in the post-pubertal group (-0.3 SD vs. -1.8 SD; p = 0.014). Within the post-pubertal group, the median BMAD Z-score was -0.2 SD in the cyclical menstruation group and -2.3 SD in the HRT group (p = 0.016). Spearman's rank correlation revealed no correlation between the BMAD Z-score and bone metabolism markers. No significant relationship was observed between the BMAD Z-score and either the vitamin D sufficiency rate or the step sufficiency rate. A negative correlation was found between BMAD Z-score and serum sclerostin in the pre-pubertal group and serum FSH in the post-pubertal group. In conclusion, the present study found no relationship between the vertebral BMAD Z-score and diet or exercise habits in TS, indicating that estrogen deficiency is the chief reason for low BMD in TS.

摘要

特纳综合征(TS)由于骨密度(BMD)低而与骨折风险增加相关。虽然已知性腺功能减退症在降低 BMD 中起作用,但其他因素尚未得到很好的研究。本研究旨在关注饮食、运动和骨代谢标志物,以确定导致 TS 患者 BMD 降低的因素。共纳入 48 名年龄在 5 至 49 岁之间的 TS 患者,包括青春期前组(n = 9)、周期性月经组(n = 6)和激素替代治疗(HRT)组(n = 33)。周期性月经组和 HRT 组统称为青春期后组。与青春期后组相比,青春期前组的骨矿物质表观密度(BMAD)Z 评分更高(-0.3 SD 比-1.8 SD;p = 0.014)。在青春期后组中,周期性月经组的中位数 BMAD Z 评分为-0.2 SD,HRT 组为-2.3 SD(p = 0.016)。Spearman 秩相关分析显示,BMAD Z 评分与骨代谢标志物之间无相关性。BMAD Z 评分与维生素 D 充足率或步幅充足率之间无显著相关性。在青春期前组中,BMAD Z 评分与血清 Sclerostin 呈负相关,在青春期后组中,BMAD Z 评分与血清 FSH 呈负相关。总之,本研究发现 TS 患者的椎体 BMAD Z 评分与饮食或运动习惯之间没有关系,这表明雌激素缺乏是 TS 患者 BMD 降低的主要原因。

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