@article {Donlevy10.1212 / WNL。0000000000207488,作者={加布里埃尔Donlevy和凯拉MD科内特和莎拉·P加内特和迷迭香害羞和盖Estilow和塞布丽娜W百胜和金伯利安德森和大卫Pareyson伊莎贝拉莫洛尼和弗朗西斯科·Muntoni玛丽·m·赖利和理查德·s·芬克尔和大卫·N·赫尔曼和凯蒂·J Eichinger和迈克尔E害羞和烧伤和约书亚Manoj P·塞斯},title ={协会的身体质量指数与儿童疾病进展与疾病}腓骨肌萎缩,elocation-id = {10.1212 / WNL。={2023}0000000000207488},年,doi = {10.1212 / WNL。出版商0000000000207488}= {Wolters Kluwer健康,公司代表美国神经病学学会},文摘={目的: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半岛投注体育官网肌萎缩(CMT)。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(体重指数> = 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(意味着CMTPedS差9.03,95 \ %可信区间0.94 - -17.12;p = 0.02),体重不足(意味着CMTPedS差5.97,95 \ %可信区间0.62 - -11.31;p = 0.02),或肥胖(意味着CMTPedS差7.96,95 \ %可信区间1.03 - -14.88;p = 0.015)表现出更大的障碍。 At 2-years, compared to being healthy weight (mean CMTPedS 17.53, SD 9.41), children who were severely underweight exhibited greater disability (mean CMTPedS difference 9.27, 95\%CI 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 {\textendash} 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.}, issn = {0028-3878}, URL = {//www.ebmtp.com/content/early/2023/06/28/WNL.0000000000207488}, eprint = {//www.ebmtp.com/content/early/2023/06/28/WNL.0000000000207488.full.pdf}, journal = {Neurology} }