@article {Donlevye717作者={加布里埃尔·a . Donlevy和凯拉医学博士科内特和萨拉·p·加内特和迷迭香害羞和盖Estilow和塞布丽娜w . Yum和金伯利安德森和大卫Pareyson和伊莎贝拉莫洛尼和弗朗西斯科·Muntoni和玛丽·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,体积={101}={7},页面= {e717——e727} = {2023}, 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={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年以上疾病首页腓骨肌萎缩(CMT)。体重指数在242年分类方法3 {\ textendash} 20岁的参与者与CMT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 \ < 17 kg / m2)、体重(体重指数> = 17 \ < 18.5 kg / m2),健康的体重(体重指数> = 18.5 \ < 25 kg / m2),超重(体重指数> = 25 \ < 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 {\ textendash} 44分,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 \ % CI 0.94 {\ textendash} 17.12;p = 0.02),体重不足(平均CMTPedS差5.97,95 \ % CI 0.62 {\ textendash} 11.31;p = 0.02),或肥胖(平均CMTPedS差7.96,95 \ % CI 1.03 {\ textendash} 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 \ % CI 0.90 {\ textendash} 17.64;p = 0.02)。2年时间,对整个样本均值CMTPedS恶化由1.72分(95 \ % CI 1.09 {\ textendash} 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 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{\textendash}10.38; p = 0.01) than those of healthy weight (mean CMTPedS change 1.79 points, 95\% CI 0.93{\textendash}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{\textendash}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}, issn = {0028-3878}, URL = {//www.ez-admanager.com/content/101/7/e717}, eprint = {//www.ez-admanager.com/content/101/7/e717.full.pdf}, journal = {Neurology} }