PT -期刊文章盟加布里埃尔·a·Donlevy盟凯拉医学博士科内特盟——莎拉·p·加内特AU -迷迭香害羞AU -盖Estilow盟-塞布丽娜w . Yum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 TI - p·梅内塞斯协会儿童身体质量指数与疾病进展的疾病援助- 10.1212 / WNL腓骨肌萎缩。0000000000207488 DP - 2023年8月15日TA -神经病首页学PG - e717 e727 VI - 101 IP - 7 4099 - //www.ez-admanager.com/content/101/7/e717.short 4100 - //www.ez-admanager.com/content/101/7/e717.full所以Neurology2023 8月15日;101 AB -背景和目的本研究的目的是评估的影响体重指数(BMI)在儿童疾病进展2年以上疾病腓骨肌萎缩(CMT)。BMI是分类方法与CMT 3岁的242名参与者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)标准。组分为体重严重不足(BMI & lt; 17 kg / m2)、体重(体重指数≥17 & lt; 18.5 kg / m2),健康的体重(体重指数≥18.5 & lt; 25 kg / m2),超重(体重指数≥25 & 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,轻微到严重)。结果基线,而个人的健康的体重(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2年,相比之下,个人的健康的体重(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, the 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 have a change in 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 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).Discussion Children with CMT who were 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 toward healthy weight may reduce disability in children with CMT.6MWT=6-minute walk test; 9HPT=9-Hole Peg Test; ANCOVA=analysis of covariance; ANOVA=analysis of variance; BMI=body mass index; CDC=Centers for Disease Control and Prevention; CMT=Charcot-Marie-Tooth disease; CMTPedS=CMT Pediatric Scale; DMD=Duchenne muscular dystrophy; IOTF=International Obesity Task Force; WHO=World Health Organization