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杜兴氏肌营养不良症儿童和青少年中,频繁未被识别的椎体骨折与体脂量增加有关。

Frequent Unrecognized Vertebral Fractures Associated with Increased Body Fat Mass in Children and Adolescents with Duchenne Muscular Dystrophy.

作者信息

Prasiw Kanlaya, Khongkhatithum Chaiyos, Fuangfa Praman, Kositwattanarerk Arpakorn, Mahachoklertwattana Pat, Poomthavorn Preamrudee

机构信息

Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

Department of Diagnostic and Therapeutic Radiology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

出版信息

Neuropediatrics. 2025 Feb;56(1):12-19. doi: 10.1055/a-2417-0441. Epub 2024 Sep 17.

Abstract

OBJECTIVE

Patients with Duchenne muscular dystrophy (DMD) have an increased risk of vertebral fractures (VFs). Ethnic variations may partly contribute to the fracture risk. This study aimed to demonstrate the VFs and body fat mass in Asian patients with DMD.

METHODS

Demographic data and DMD-related parameters of the enrolled patients were collected. Lateral thoracolumbar spine radiographs were performed for VF assessment. The Genant classification was applied for VF severity grading (mild, moderate, and severe). Body composition analysis using dual-energy X-ray absorptiometry was performed. Serum calcium, phosphate, intact parathyroid hormone, and 25-hydroxyvitamin D concentrations were determined.

RESULTS

There were 25 children and adolescents with DMD enrolled. The median (interquartile range [IQR]) age was 12.9 (9.6-19.3) years. Nine patients (36%) had VFs with a total of 31 sites of VFs (mild,  = 10; moderate,  = 3; and severe,  = 18). These VFs had never been recognized prior to this study. Comparing with the non-VF group, the VF group received a significantly greater cumulative prednisolone equivalent dose (1,258 [948-1,664] vs. 291 [17-823] mg/kg,  0.003). Body fat mass, represented by fat mass index and body fat percentage -scores, was greater in the VF group (2.46 [2.21-2.51] vs. 1.63 [0.36-2.07],   0.011 and 4.4 [3.1-5.5] vs. 1.8 [0.6-3.5],   0.008, respectively). No differences in serum calciotropic hormones and vitamin D status were demonstrated between patients with and without VFs.

CONCLUSIONS

VFs were frequent in patients with DMD. Patients with VFs had greater cumulative glucocorticoid dose and body fat mass than those without VFs.

摘要

目的

杜氏肌营养不良症(DMD)患者发生椎体骨折(VF)的风险增加。种族差异可能部分导致骨折风险。本研究旨在证明亚洲DMD患者的椎体骨折情况和体脂量。

方法

收集入组患者的人口统计学数据和与DMD相关的参数。进行胸腰椎侧位X线片以评估椎体骨折。采用Genant分类法对椎体骨折严重程度进行分级(轻度、中度和重度)。使用双能X线吸收法进行身体成分分析。测定血清钙、磷、完整甲状旁腺激素和25-羟基维生素D浓度。

结果

共纳入25例患有DMD的儿童和青少年。中位(四分位间距[IQR])年龄为12.9(9.6 - 19.3)岁。9例患者(36%)发生椎体骨折,共31处椎体骨折部位(轻度 = 10处;中度 = 3处;重度 = 18处)。这些椎体骨折在本研究之前从未被发现。与无椎体骨折组相比,椎体骨折组接受的累积泼尼松龙等效剂量显著更高(1258 [948 - 1664] 与291 [17 - 823] mg/kg,P = 0.003)。以脂肪量指数和体脂百分比评分表示的体脂量在椎体骨折组中更高(分别为2.46 [2.21 - 2.51] 与1.63 [0.36 - 2.07],P = 0.011;以及4.4 [3.1 - 5.5] 与1.8 [0.6 - 3.5],P = 0.008)。有椎体骨折和无椎体骨折患者之间血清钙调节激素和维生素D状态无差异。

结论

DMD患者中椎体骨折很常见。有椎体骨折的患者比无椎体骨折的患者累积糖皮质激素剂量更高且体脂量更大。

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