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杜氏肌营养不良症患者的骨矿物质密度和维生素D水平与类固醇使用及步行能力的关系。

The relationship of bone mineral density and vitamin D levels with steroid use and ambulation in patients with Duchenne muscular dystrophy.

作者信息

Sertpoyraz Filiz Meryem, Tiftikçioğlu Bedile İrem

机构信息

Department of Physical Medicine and Rehabilitation, Health Science University, Izmir Tepecik Training and Research Hospital, Izmir, Turkey.

Department of Neurology, Health Science University, Izmir Tepecik Training and Research Hospital, Izmir, Turkey.

出版信息

Turk J Phys Med Rehabil. 2019 Aug 20;65(3):216-221. doi: 10.5606/tftrd.2019.3565. eCollection 2019 Sep.

Abstract

OBJECTIVES

This study aims to assess the bone mineral density (BMD) and serum levels of 25(OH)-vitamin D and their relationship with steroid use and ambulation in patients with Duchenne muscular dystrophy (DMD).

PATIENTS AND METHODS

Between January 2017 and May 2018, medical records of a total of 67 male patients (mean age, 13.9±4.3 years; range, 8 to 25 years) who were diagnosed with definite DMD were retrospectively analyzed. Demographic data, functional activity level, steroid use, fracture history and location, serum levels vitamin D, and lumbar and hip Z-scores in BMD at the time of the initial admission were recorded.

RESULTS

The mean level of vitamin D was 13.4±7.5 ng/mL. In terms of serum levels of vitamin D, 28 patients (41.8%) had severe deficiency, 31 (46.3%) had insufficiency, and five patients (7.5%) had deficiency. Only three (4.5%) of the patients had sufficient levels of vitamin D. The hip Z-scores were significantly lower than lumbar Z scores. There was no significant difference in the lumbar and hip BMD measurements between the patients with and without steroid use. Lumbar Z-scores were significantly lower in non-ambulatory patients than ambulatory patients.

CONCLUSION

It is of utmost importance to evaluate the initial serum vitamin D levels in terms of bone health and prescribe replacement in case of deficiency/insufficiency in DMD patients. Since the decrease in the BMD is evident in this patient population, maintaining the mobilization as long as possible, providing loading on the bone for a long time, may be beneficial.

摘要

目的

本研究旨在评估杜氏肌营养不良症(DMD)患者的骨矿物质密度(BMD)、血清25(OH)-维生素D水平,以及它们与类固醇使用和行走能力的关系。

患者与方法

回顾性分析2017年1月至2018年5月期间共67例确诊为DMD的男性患者(平均年龄13.9±4.3岁;范围8至25岁)的病历。记录初始入院时的人口统计学数据、功能活动水平、类固醇使用情况、骨折病史及部位、血清维生素D水平以及腰椎和髋部BMD的Z值。

结果

维生素D的平均水平为13.4±7.5 ng/mL。就血清维生素D水平而言,28例患者(41.8%)严重缺乏,31例(46.3%)不足,5例患者(7.5%)缺乏。只有3例(4.5%)患者维生素D水平充足。髋部Z值显著低于腰椎Z值。使用类固醇和未使用类固醇的患者在腰椎和髋部BMD测量上无显著差异。非行走患者的腰椎Z值显著低于行走患者。

结论

从骨骼健康角度评估DMD患者初始血清维生素D水平并在缺乏/不足时进行补充至关重要。鉴于该患者群体中BMD下降明显,尽可能长时间保持活动,让骨骼长期承受负荷,可能有益。

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