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2
Vitamin D in corticosteroid-naïve and corticosteroid-treated Duchenne muscular dystrophy: what dose achieves optimal 25(OH) vitamin D levels?初治和接受皮质类固醇治疗的杜氏肌营养不良症患者中的维生素D:何种剂量能达到最佳的25(OH)维生素D水平?
Arch Dis Child. 2016 Oct;101(10):957-61. doi: 10.1136/archdischild-2015-308825. Epub 2016 May 31.
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Fracture in Duchenne Muscular Dystrophy: Natural History and Vitamin D Deficiency.杜氏肌营养不良症中的骨折:自然病史与维生素D缺乏症
J Child Neurol. 2016 Aug;31(9):1181-7. doi: 10.1177/0883073816650034. Epub 2016 May 24.
4
Risk Factors for First Fractures Among Males With Duchenne or Becker Muscular Dystrophy.杜氏或贝克型肌营养不良男性首次骨折的危险因素
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Bone health in children with neuromuscular disorders.神经肌肉疾病患儿的骨骼健康
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Insights into bone health in Duchenne muscular dystrophy.杜氏肌营养不良症患者骨骼健康的相关见解。
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Bone health and associated metabolic complications in neuromuscular diseases.神经肌肉疾病中的骨骼健康及相关代谢并发症
Phys Med Rehabil Clin N Am. 2012 Nov;23(4):773-99. doi: 10.1016/j.pmr.2012.08.005. Epub 2012 Oct 17.
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Bone health in boys with Duchenne Muscular Dystrophy on long-term daily deflazacort therapy.长期每日使用地夫可特治疗的杜氏肌营养不良症男孩的骨骼健康。
Neuromuscul Disord. 2012 Dec;22(12):1040-5. doi: 10.1016/j.nmd.2012.06.354. Epub 2012 Jul 21.
10
Bone mass development in patients with Duchenne and Becker muscular dystrophies: a 4-year clinical follow-up.杜氏肌营养不良症和贝克肌营养不良症患者的骨量发育:4 年临床随访。
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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.

DOI:10.5606/tftrd.2019.3565
PMID:31663069
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6797922/
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。