遗传性肝酶缺陷可引起糖原代谢紊乱,导致糖原分解、糖酵解及肝肾中糖生成困难,因而发生低血糖,能量产生低,糖原累积。故肝肿大、肌肉无力,生长发育不良,重症者多于婴儿期死亡。由于不同酶的缺陷,可造成十型糖原代谢病,其中Ⅰ、Ⅲ、Ⅵ、Ⅸ型可发生低血糖,其特点如下: 1.糖原代谢病Ⅰ型:又称肝型,因1929年首先 Hereditary liver enzymes can cause glycogen metabolism disorders, resulting in glycogenolysis, glycolytic and liver and kidney sugar production difficulties, resulting in hypoglycemia, low energy production, glycogen accumulation. Therefore, hepatomegaly, muscle weakness, poor growth and development, severe cases more than infancy death. Because of different enzyme defects, can cause ten glycogen metabolism disease, of which Ⅰ, Ⅲ, Ⅵ, Ⅸ hypoglycemia can occur, its features are as follows: 1. Glycogen metabolism type Ⅰ: also known as liver type, due to 1929 First of all