脊柱肌肉缩的儿童的骨健康状况
Joanna Yuet-Ling Tung1, Tsz-Kit Chow2, Monique Wai3
1Department of Paediatrics and Adolescent Medicine, Hong Kong Children's Hospital, Hong Kong SAR, China.
Journal of bone metabolism
|December 11, 2023
概括
脊髓肌肉缩 (SMA) 的儿童通常具有低骨矿物质密度 (BMD) 和骨折. 然而,目前的骨质疏松症定义可能没有完全捕捉到这一群体的骨健康风险.
科学领域:
- 儿科神经肌肉疾病 儿科神经肌肉疾病
- 骨代谢和健康 骨代谢和健康
- 罕见的遗传疾病 罕见的遗传疾病
背景情况:
- 脊髓肌肉缩 (SMA) 是一组罕见的,遗传的神经肌肉疾病.
- 骨健康通常是SMA儿童的一个被忽视的问题.
- 这项研究评估了香港患有SMA的儿童的骨健康.
研究的目的:
- 评估SMA儿童的骨健康状况.
- 评估骨折史,骨矿物质密度 (BMD) 和维生素D水平.
- 根据当前的定义来确定骨质疏松症的流行率.
主要方法:
- 对来自两个香港中心的32名SMA儿童进行了回顾性研究.
- 通过骨折史,双能X射线吸收度 (BMD) 和血清25-基维生素D (25[OH]D) 来评估骨健康.
- 分析了SMA类型,年龄,性别,行走,25(OH) D和骨健康结果之间的关联.
主要成果:
- 在17名被评估的患者中,16名患者观察到低BMD.
- 四名患者有长骨折史,并接受过双酸盐治疗.
- 没有发现临床因素与骨折史或BMD Z-score之间有显著的关联. 只有一个患者符合2019年ISCD儿科骨质疏松症定义.
结论:
- 接受疾病修饰治疗的SMA儿童经常表现出低BMD和骨折史.
- 根据2019年ISCD儿科定义,骨质疏松症并不常见.
- 对于这种高风险的儿科患者群体,骨质疏松症的专门定义可能是必要的.
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