Ⅱ型糖原蓄积病是一种比较少见的代谢障碍性疾病。根据发病的迟早 ,分为婴儿型及迟发型[1,2 ,3] 。在迟发型中 ,根据发病年龄不同 ,又可分儿童型及成人型。在婴儿型 ,全身多种组织有糖原沉积 ,尤其是心肌、骨骼肌及肝脏受累最严重。在儿童型 ,除骨骼肌受累外 ,心、肝等脏器是否 Type Ⅱ glycogen storage disease is a relatively rare metabolic disorder. According to the onset of sooner or later, divided into infants and delayed type [1,2,3]. In delayed type, according to the age of onset is different, but also can be divided into children and adults. In infants, the body a variety of organizations have glycogen deposition, especially myocardial, skeletal muscle and liver involvement most serious. In children, in addition to skeletal muscle involvement, heart, liver and other organs is