肌强直性营养不良患者的区域性身体成分和功能障碍。
Regional body composition and functional impairment in patients with myotonic dystrophy.
机构信息
Centre de Référence des Maladies Neuromusculaires, Médecine Interne Orientée vers les Maladies Orphelines et Systémiques, Tour Drouet, CHU de Nancy, Hôpitaux de Brabois, 54511 Vandoeuvre Cedex, France.
出版信息
Muscle Nerve. 2011 Oct;44(4):503-8. doi: 10.1002/mus.22099. Epub 2011 Aug 8.
INTRODUCTION
In this study we determined regional body composition in myotonic dystrophy (DM1) and able-bodied controls and evaluated the relationship between fat and lean tissue mass and functional impairment in DM1 patients.
METHODS
Dual-energy X-ray absorptiometry (DEXA) was used to obtain regional measurements of fat-free mass index (FFMI) and fat mass index (FMI) in 48 DM1 and anthropometrically matched control pairs.
RESULTS
DM1 patients had lower regional FFMI and higher FMI than controls (P < 0.01-0.001). In DM1 patients, total FMI increased significantly with increased muscular disability rating, decreased motor function measurement, and with both decreasing vital capacity and total lung capacity. Hypertriglyceridemia correlated with increasing FMI.
CONCLUSIONS
Regional FFMI is decreased in DM1, whereas FMI is underestimated by body mass index and is negatively correlated with patients' functional capacity. DEXA may provide valuable supporting evidence in the management of DM1.
简介
在这项研究中，我们确定了肌强直性营养不良（DM1）患者和健康对照组的区域性身体成分，并评估了 DM1 患者的脂肪和瘦组织质量与功能障碍之间的关系。
方法
使用双能 X 射线吸收法（DEXA）对 48 对 DM1 患者和人体测量匹配的对照组进行无脂肪质量指数（FFMI）和脂肪质量指数（FMI）的区域性测量。
结果
DM1 患者的区域性 FFMI 低于对照组，FMI 高于对照组（P < 0.01-0.001）。在 DM1 患者中，总 FMI 随着肌肉残疾评分的增加、运动功能测量的减少以及肺活量和总肺容量的减少而显著增加。高甘油三酯血症与 FMI 的增加相关。
结论
DM1 患者的区域性 FFMI 降低，而 FMI 被身体质量指数低估，并与患者的功能能力呈负相关。DEXA 可能为 DM1 的管理提供有价值的辅助证据。
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