在患有基尿症的年轻成年人中,低骨矿物质密度的高患病率
Kısmet Çıkı1, Ayça Burcu Kahraman1, Halil Tuna Akar1
1Division of Pediatric Metabolism, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Postgraduate medicine
|December 18, 2024
概括
低骨矿物质密度 (BMD) 影响了25%的成年人患有经典尿症 (cPKU). 男性性别和低体重指数 (BMI) 被确定为该人群中 BMD 降低的关键风险因素.
科学领域:
- 代谢障碍 代谢障碍 代谢障碍
- 骨健康 骨健康 骨健康
- 营养科学 营养科学
背景情况:
- 在古典的基尿症 (cPKU) 中,限制氨酸 (Phe) 的饮食可能会对骨健康产生负面影响.
- 关于CPKU成年人骨矿物质密度 (BMD) 的数据有限,有相互矛盾的报道.
- 这项研究评估了成年cPKU患者队列中的骨质疏松症和相关风险因素.
研究的目的:
- 评估成年人诊断出古典尿症 (cPKU) 的骨矿物质密度 (BMD).
- 为了确定在这个患者群体中导致低BMD的特定风险因素.
- 在cPKU.KU中为骨健康提供临床监测和管理策略的信息.
主要方法:
- 研究了80名患有cPKU的成年患者 (18.3-39.4岁).
- 在股骨和腰椎的骨矿物质密度 (BMD) 用双能X射线吸收计测量.
- 患者被分为低BMD (Z-score ≤-2) 和正常的BMD (Z-score > -2) 组进行分析.
主要成果:
- 在25%的成人cPKU队列中观察到低的BMD.
- 低BMD组表现出男性患病率更高 (75%vs. 40%) 和较低的平均体重指数 (BMI).
- 在低BMD组中观察到和维生素D水平升高,尽管很小;年龄和Phe水平不是显著的因素.
结论:
- 这项研究证实,男性性别和低BMI是CPKU成年人低BMD的重要危险因素.
- 低BMD甚至在患有cPKU的年轻人中也很普遍,无论年龄,Phe水平或补充剂.
- 对年轻成人cPKU患者,特别是男性和低BMI患者来说,定期的骨健康评估至关重要.
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