肝糖原累积症是一种较罕见的遗传代谢疾病.由于酶缺陷所造成的先天性糖原代谢紊乱,可累及各器官,如肝、肌肉、心、肾、胃肠及脑等.幼年患者多以肝受累为主,年长儿则以肌肉受累为主.1984年,我院发现两例报告如下: 例1(见封3图1),男14岁,生后6个月发现肝大.3岁会站立,6岁独走,常跌跤.9岁开始上学,至今读5年级,学习较差. Hepatic glycogenosis is a rare inherited metabolic disease.Due to the congenital glycogen metabolism disorders caused by enzyme defects, it can affect various organs such as liver, muscle, heart, kidney, gastrointestinal and brain, etc. Juvenile patients In 1984, our hospital found two cases reported as follows: Example 1 (see seal 3 Figure 1), male 14 years old, 6 months after birth, found that liver Standing at the age of 3, standing alone at the age of 6, she often fell and fell, starting school at the age of 9 and now attending the 5th grade with poor learning.