脊髓性肌萎缩症患儿营养管理研究进展

doi:10.12372/jcp.2022.21e1244

摘要/Abstract

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

Abstract:

Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease caused by variation in survival motor neuron 1 (SMN1) 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 problems and nutritional intervention for SMA children.

Key words: spinal muscular atrophy, child, nutrition management

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.

WANG Wenqiao, MAO Shanshan, MA Ming. Research progress in nutritional management of children with spinal muscular atrophy[J]. Journal of Clinical Pediatrics, 2022, 40(3): 235-240.

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