评估身体成分作为脊髓性肌萎缩症的潜在生物标志物。
Evaluation of body composition as a potential biomarker in spinal muscular atrophy.
机构信息
Developmental Neurology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
The Dubowitz Neuromuscular Centre, UCL NIHR GOSH Biomedical Research Centre, UCL Great Ormond Street Institute of Child Health, London, UK.
出版信息
Muscle Nerve. 2020 Apr;61(4):530-534. doi: 10.1002/mus.26823. Epub 2020 Feb 7.
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.
简介
我们旨在研究身体成分（BC）与脊髓性肌萎缩症（SMA）特定运动功能评估之间的相关性。
方法
本横断面研究招募了 1 至 10 岁的 SMA 类型 I 或 II 型患者。该方案包括人体测量学测量以及双能 X 射线吸收法，以评估脂肪量（FM）、瘦体重（LM）、无脂肪量（FFM）、FM 和 FFM 指数（FMI、FFMI），以及运动功能评估（费城儿童医院婴儿神经肌肉疾病 SMAI 测试和哈默史密斯功能运动扩展 SMAII 测试）。
结果
共纳入 88 名儿童。所有儿童的 FM 百分比均高于参考值。SMAI 中，运动功能与体重指数（BMI）、FFMI 和 LMI 中度相关，与 SMAII 中的 FFMI、LMI 和 LM：FM 比值弱相关。
讨论
BC 有望成为 SMA 的潜在生物标志物，但需要进一步研究。
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