在13例糖原累积病,Cori 分型的Ⅰ、Ⅳ、Ⅵ及Ⅸ型患者中,观察了临床出血情况,作了出血时间、血小板粘着性、凝血酶原消耗试验、血小板聚集试验(包括用二磷酸腺苷、胶原组织、肾上腺素等诱发聚集)等血小板功能的检查。结果发现只在7例伴有萄葡糖-6-磷酸酶缺陷的患者(Ⅰ型)中,显示有血小板功能损害。在临床表现上易有外伤出血,常有鼻 In 13 cases of glycogen storage disease, Cori classification Ⅰ, Ⅳ, Ⅵ and Ⅸ type of patients, the clinical bleeding was observed, bleeding time, platelet adhesion, prothrombin depletion test, platelet aggregation test (including the use of Adenosine diphosphate, collagen, epinephrine induced aggregation) and other platelet function tests. The results showed that in only 7 patients with defects in glucose-6-phosphatase (type I) showed platelet dysfunction. On the clinical manifestations of traumatic bleeding, often nose