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| 脊髓性肌萎缩症患儿营养管理研究进展 | |
| 引用本文： | 王文俏,毛姗姗,马鸣.脊髓性肌萎缩症患儿营养管理研究进展[J].临床儿科杂志,2022,40(3):235-240. |
| 作者姓名： | 王文俏 毛姗姗 马鸣 |
| 作者单位： | 1.浙江大学医学院附属儿童医院临床营养科 国家儿童健康与疾病临床医学研究中心 2.浙江大学医学院附属儿童医院神经内科 国家儿童健康与疾病临床医学研究中心（浙江杭州 310052） |
| 基金项目： | 浙江省基础公益分析测试项目(No.GC21H090004)。 |
| 摘 要： | 脊髓性肌萎缩症(SMA)是一种严重的神经肌肉疾病，因运动神经元存活基因1(SMN1)缺陷引起。由于肌肉无力、活动量少、消化道症状以及吞咽困难等原因，患儿发生营养不足和营养过剩的概率常明显高于正常儿童，因此SMA的营养管理至关重要。文章就SMA患儿的营养评估、可能出现的营养问题以及相关营养干预策略进行阐述。 |
| 关 键 词： | 脊髓性肌萎缩 儿童 营养管理 |
| 收稿时间： | 2021-08-30 |
Research progress in nutritional management of children with spinal muscular atrophy |
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| WANG Wenqiao,MAO Shanshan,MA Ming.Research progress in nutritional management of children with spinal muscular atrophy[J].The Journal of Clinical Pediatrics,2022,40(3):235-240. | |
| Authors: | WANG Wenqiao MAO Shanshan MA Ming |
| Institution: | 1. Department of Clinical Nutrition, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health 2. Department of Neurology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, 310052, Zhejiang, China |
| Abstract: | Spinal muscular atrophy(SMA)is an autosomal recessive neurodegenerative disease caused by variation in survival motor neuron 1(SMN 1)gene.Due to poor muscle reserves,low physical activity,digestive symptoms and dysphagia,the incidence of undernutrition and overnutrition in SMA children is often significantly higher than that in normal children.Therefore,nutritional management is essential for children with SMA.This paper reviewed the nutritional evaluation,related nutritional proble ms and nutritional intervention for SMA children. |
| Keywords: | spinal muscular atrophy child nutrition management |
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