RT期刊文章SR电子T1协会与儿童身体质量指数与疾病进展的神经学神经学疾病摩根富林明乔腓骨肌萎缩FD Lippincott Williams &威尔金斯10.1212 SP / WNL。半岛投注体育官网0000000000207488 10.1212 / WNL。0000000000207488 A1加布里埃尔Donlevy A1凯拉MD科内特A1莎拉P加内特A1迷迭香害羞A1盖Estilow A1塞布丽娜W百胜A1金伯利安德森A1 (Davide Pareyson A1伊莎贝拉莫罗尼A1 Francesco Muntoni A1玛丽·m·赖利A1理查德·s·芬克尔A1大卫·N·赫尔曼A1凯蒂J Eichinger A1迈克尔E害羞A1约书亚烧伤A1 Manoj P·塞斯年2023 UL //www.ebmtp.com/content/early/2023/06/28/WNL.0000000000207488.abstract AB目的:这项研究的目的是评估的影响体重指数(BMI)在疾病进展在中国开放儿童疾病腓骨肌萎缩(CMT)。半岛投注体育官网方法:BMI与CMT分类242名参与者3年来参与遗传神经病变的财团,使用国际肥胖特别工作组(基于成人BMI值,kg / m2)。组归类为体重严重不足(BMI < 17 kg / m2)、体重(体重指数≥17 kg / m2 < 18.5 kg / m2),健康的体重(体重指数≥18.5 kg / m2 < 25 kg / m2),超重(体重指数≥25 kg / m2 < 30 kg / m2)和肥胖(BMI≥30 kg / m2)。使用CMT儿科疾病严重度评估量表(CMTPedS)的临床结果评估残疾(0-44点,mild-severe)。结果:基线,而被健康的体重(9.22意味着CMTPedS 15.48, SD),孩子体重严重不足(平均CMTPedS差9.03,95%可信区间0.94 - -17.12;p = 0.02),体重不足(平均CMTPedS差5.97,95%可信区间0.62 - -11.31;p = 0.02),或肥胖(平均CMTPedS差7.96,95%可信区间1.03 - -14.88;p = 0.015)表现出更大的障碍。在中国开放,而被健康的体重(9.41意味着CMTPedS 17.53, SD),孩子体重严重不足表现出更大的残疾(平均CMTPedS差9.27,95%可信区间0.90 - -17.64; p=0.02). Over the 2-year periods, mean CMTPedS for the whole sample deteriorated by 1.72 points (95%CI 1.09-2.38; p<0.001), with severely underweight children progressing at the fastest rate (mean CMTPedS change of 2.3, 95%CI 1.53 – 6.13; p=0.21). In children who did not change BMI categories over 2-years (69% of sample), CMTPedS scores deteriorated faster in those who were severely underweight (mean CMTPedS change 6.40 points 95%CI 2.42 -10.38; p=0.01) than those of a healthy weight (mean CMTPedS change 1.79 points 95%CI 0.93 - 2.69; p<0.001). For children who changed BMI categories (31% of sample), CMTPedS scores deteriorated faster in children who became overweight/obese (mean CMTPedS change 2.76 points 95%CI 0.11 - 5.41; p=0.031).Conclusion: Children with CMT who severely underweight, underweight, or obese exhibited greater disability at baseline. Over the 2-year period in those whose BMI remained stable, severely underweight children deteriorated at the fastest rate. For children who changed BMI categories over the 2 years, CMTPedS scores deteriorated faster in children who became overweight/obese. Interventions that maintain or improve BMI towards a healthy weight may reduce disability in children with CMT.