中国实用儿科杂志

中国实用儿科杂志 ›› 2022, Vol. 37 ›› Issue (10): 748-754.DOI: 10.19538/j.ek2022100608

• 专题笔谈 • 上一篇    下一篇

脊髓性肌萎缩症患者的营养管理

  

  1. 1.北京大学第一医院  a 临床营养科，b 儿科，北京  100034；2.北京协和医院临床营养科，北京  100730
  • 出版日期:2022-10-06 发布日期:2022-11-11
  • 通讯作者: 窦攀，电子信箱：doupan@pkufh.cn
  • 基金资助:
    中央高水平医院临床科研业务费资助北京大学第一医院高质量临床研究专项［2022CR69］

Nutritional management in patients with spinal muscular atrophy

  1. *Department of Clinical Nutrition，First Hospital of Peking University，Beijing  100034，China
  • Online:2022-10-06 Published:2022-11-11

摘要： 脊髓性肌萎缩（spinal muscular atrophy，SMA）是一种严重的常染色体隐性遗传病，积极的营养治疗可改善SMA患者的营养代谢和体成分，提高药物疗效，保护骨骼及器官功能，提高生存质量及存活率。文章结合国内外经验，分析SMA患者的常见营养相关合并症，如体重和体成分的异常、吞咽障碍、胃排空延迟、胃食管反流和便秘、骨密度下降、维生素和矿物质缺乏、脂肪代谢紊乱、糖代谢紊乱，探讨营养诊断和干预对策，希望SMA患者的营养管理能得到更多的医生、患者及家属的关注。

关键词: 脊髓性肌萎缩, 营养, 代谢, 干预

Abstract: Spinal muscular atrophy（SMA）is a severe autosomal recessive inherited disease. Active nutritional th-erapy can improve nutritional metabolism and body composition of SMA patients，improve drug efficacy，protect bone and organ functions，and improve the quality of life and survival rate. In this study，based on the experience at home and abroad，the common nutrition-related complications of SMA patients were analyzed，such as abnormal weight and body composition，dysphagia，delayed gastric emptying，gastroesophageal reflux，constipation，decreased bone density，vitamin and mineral deficiencies，and disorders of carbohydrate or lipid metabolism. Nutritional diagnosis and intervention strategies were studied and it is hoped that the nutritional management of SMA patients can obtain more attention from doctors，patients and their families.

Key words: spinal muscular atrophy, nutrition, metabolism, intervention