Abstract: Glycogen storage diseases（GSD）is a group of abnormal glycogen metabolism caused by congenital enzyme defects，and glycogen accumulation in the liver，muscle，myocardial tissue leads to a variety of diseases. Most of them lack specific treatment.Diet and nutritional intervention can improve the metabolic disorders of patients with glycogen storage diseases，ensure the growth and development of patients，and improve the quality of life.Attention should be paid to the long-term nutritional management of glycogen storage diseases.

Key words: glycogen storage disease, diet, nutritional intervent

摘要： 糖原累积病（glycogen storage disease，GSD）是由于先天性酶缺陷所造成的一组糖原代谢异常，患儿肝脏、肌肉、心肌组织内糖原累积，导致多种疾病，大多缺乏特效治疗，饮食和营养干预可改善部分糖原累积病患者的代谢紊乱，保证患者的生长发育，提高生存质量。应重视糖原累积病的长期营养管理。

关键词: 糖原累积病, 饮食, 营养干预