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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。

Bone mass development in patients with Duchenne and Becker muscular dystrophies: a 4-year clinical follow-up.

机构信息

Department of Orthopaedics, Institute of Clinical Sciences, Sahlgrenska University Hospital/Östra, Sahlgrenska Academy at the University of Gothenburg, Göteborg, Sweden.

出版信息

Acta Paediatr. 2012 Apr;101(4):424-32. doi: 10.1111/j.1651-2227.2011.02532.x. Epub 2011 Dec 9.

Abstract

AIM

To investigate the longitudinal development of bone mass in patients with Duchenne and Becker muscular dystrophies and to study the impact of muscle strength and motor function on bone mass in these patients.

METHODS

Eighteen patients with Duchenne muscular dystrophy (2.3-19.7 years at baseline) and six patients with the milder Becker muscular dystrophy (10.8-18.9 years at baseline) were followed during a 4-year period with respect to areal bone mineral density (BMD), motor function and muscle strength.

RESULTS

Greater bone mineral accretion was observed in the Becker patient group compared with the age-related Duchenne group above 10 years of age, and the older patients with Duchenne experienced decreased femoral neck BMD during the study period. In the study group, significant correlations were found between BMD in the lower extremities and muscle function parameters.

CONCLUSIONS

The differences in BMD between patients with Duchenne and Becker as well as between different bone measurement sites demonstrated in the present study point out the importance of preserving muscle strength and motor function in patients with muscular dystrophy. Moreover; it highlights the value of performing region-specific analysis of the bone quality in these patients.

摘要

目的

研究杜兴氏肌营养不良症和贝克型肌营养不良症患者的骨量纵向发育情况,并研究肌肉力量和运动功能对这些患者骨量的影响。

方法

18 名杜兴氏肌营养不良症患者(基线时年龄为 2.3-19.7 岁)和 6 名轻度贝克型肌营养不良症患者(基线时年龄为 10.8-18.9 岁)在 4 年的随访期间接受了骨矿物质密度(BMD)、运动功能和肌肉力量的评估。

结果

与年龄相关的杜兴氏肌营养不良症患者相比,10 岁以上的贝克患者组观察到更大的骨矿物质积累,研究期间,年龄较大的杜兴氏肌营养不良症患者出现股骨颈 BMD 下降。在研究组中,下肢 BMD 与肌肉功能参数之间存在显著相关性。

结论

本研究中观察到的杜兴氏肌营养不良症患者和贝克型肌营养不良症患者之间以及不同骨测量部位之间的 BMD 差异,指出了在肌营养不良症患者中保持肌肉力量和运动功能的重要性。此外,它强调了对这些患者进行特定区域的骨质量分析的价值。

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