Evaluation of body composition as a potential biomarker in spinal muscular atrophy
Evaluation of body composition as a potential biomarker in spinal muscular atrophy
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DOI：
10.1002/mus.26823
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发表时间：
2020-02-07
期刊：
影响因子：
3.4
通讯作者：
Bertoli, Simona
中科院分区：
文献类型：
--
作者：
Baranello, Giovanni;De Amicis, Ramona;Bertoli, Simona
简介 我们的目的是调查身体成分 (BC) 和脊髓性肌萎缩症 (SMA) 特异性运动功能评估之间的相关性。方法 本横断面研究招募了年龄 1 至 10 岁的 SMA 类型 I 或 II 患者。该方案包括人体测量和双能 X 射线吸收测量法，以评估脂肪量 (FM)、瘦体重 (LM)、去脂体重 (FFM)、FM 和 FFM 指数 (FMI、FFMI) 和运动功能评估（费城儿童医院婴儿神经肌肉疾病测试量表 SMAI 和 Hammersmith 功能运动量表？针对 SMAII 扩展）。 结果 88 名儿童包括在内。所有的 FM 百分比均高于参考值。 SMAI 中的运动功能与体重指数 (BMI)、FFMI 和 LMI 呈中度相关，而与 SMAII 中的 FFMI、LMI 和 LM:FM 比率呈弱相关。 讨论 BC 有希望作为 SMA 的潜在生物标志物，但需要进一步研究。
Introduction We aimed to investigate the correlation between body composition (BC) and spinal muscular atrophy (SMA)-specific motor function assessments.Methods Patients with SMA types I or II, aged 1 to 10 years, were recruited in this cross-sectional study. The protocol included anthropometric measurements, and dual-energy X-ray absoprtiometry to assess fat mass (FM), lean mass (LM), fat-free mass (FFM), FM and FFM indexes (FMI, FFMI), and motor function assessments (Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scale for SMAI, and Hammersmith Functional Motor Scale?Expanded for SMAII).Results Eighty-eight children were included. All had a higher FM percentage than reference values. Motor function was moderately correlated with body mass index (BMI), FFMI, and LMI in SMAI, and weakly correlated with FFMI, LMI, and LM:FM ratio in SMAII.Discussion BC shows promise as a potential biomarker for SMA, but further studies are needed.