中国实用儿科杂志

中国实用儿科杂志 ›› 2022, Vol. 37 ›› Issue (10): 743-748.DOI: 10.19538/j.ek2022100607

• 专题笔谈 • 上一篇    下一篇

线粒体病的营养干预

  

  1. 国家儿童医学中心（北京）  首都医科大学附属北京儿童医院  a 临床营养科，b 神经内科，北京  100045
  • 出版日期:2022-10-06 发布日期:2022-11-11
  • 通讯作者: 方方，电子信箱：fangfang@bch.com.cn

Nutritional intervention in mitochondrial diseases

  1. *Department of Clinical Nutrition，Beijing Children’s Hospital，Capital Medical University，National Center for Children’s Health，Beijing  100045，China
  • Online:2022-10-06 Published:2022-11-11

摘要： 线粒体病是一组罕见的多系统疾病，目前尚无有效的治疗方法，多学科的对症支持十分必要。线粒体病患者营养摄入不足，营养不良又可继发线粒体功能障碍并加重病情。营养评估及保证营养摄入是所有治疗的基础，此外，营养补充剂、生酮饮食等还可以改善或稳定疾病症状。因此，线粒体病医学专家和临床营养师应共同努力，提高患者的生活质量。

关键词: 线粒体病, 营养干预, 营养补充剂, 生酮饮食

Abstract: Mitochondrial diseases are a group of rare multisys-tem disorders. There are no effective therapies and multidisciplinary symptomatic support is necessary.Patients with mitochondrial diseases have an inadequate nutrition intake，and malnutrition may cause secondary mitochondrial dysfunction and aggravate the disease. Evaluating the nutritional status and providing an adequate nutrition should be at the basis of any medical interventions. Moreover，nutritional supplements，ketogenic diet，etc. may improve，or stabilize，disease signs and symptoms. For this reason，medical experts in mitochondrial diseases and clinical nutritionists should work together to improve the quality of life of the patients.

Key words: mitochondrial diseases, nutritional intervention, nutritional supplements, ketogenic diet