TY -的T1 -协会儿童身体质量指数与疾病进展的神经学疾病摩根富林明腓骨肌萎缩——乔-神经学做- 10.1212 / WNL。半岛投注体育官网0000000000207488 SP - 10.1212 / WNL。0000000000207488 AU -加布里埃尔Donlevy盟——凯拉MD科内特盟——莎拉·P加内特AU -迷迭香害羞AU -盖Estilow盟-塞布丽娜W百胜盟金伯利安德森AU -大卫。Pareyson盟伊莎贝拉莫罗尼AU -弗朗西斯科·Muntoni盟-玛丽·m·赖利AU -理查德·s·芬克尔AU -大卫·N·赫尔曼盟凯蒂J Eichinger AU -迈克尔E害羞盟约书亚烧伤盟Manoj P·塞斯Y1 - 2023/06/28 UR - //www.ebmtp.com/content/early/2023/06/28/WNL.0000000000207488.abstract N2 -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 & lt; 18.5 kg / m2),健康的体重(体重指数≥18.5 kg / m2 & lt; 25 kg / m2),超重(体重指数≥25 kg / m2 & lt; 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. ER -