METABOLISM IN CHILDREN WITH GLYCOGEN STORAGE DISEASE, TYPE I

这项研究的目的是确定葡萄糖的产生率 葡萄糖-6-磷酸酶缺陷型、葡萄糖-6缺乏型患者肝脏 磷酸酶和缺乏性转位酶I以及III型糖原增多症。 有报道称，I型糖原沉积症患者的肝脏 产生一些葡萄糖。这一解释需要经过测试才能确定 肝脏产生的葡萄糖是否存在可检测到的差异 在完全缺乏葡萄糖-6-磷酸酶的个体中 与有部分缺陷的人形成对比。同样，有没有一个 转移酶I缺陷患者与葡萄糖6患者的差异 磷酸盐缺陷？肝脏葡萄糖的产生是否存在以下差异 转位酶I缺陷的患者在以下情况下表现较轻 与受影响更严重的人相比？

This study was designed to determine the rate of glucose production by the liver in patients with absent glucose 6 phosphatase, deficient glucose 6 phosphatase and deficient translocase I as well as type III glycogenosis. There are reports that the liver of patients with type I glycogenosis produce some glucose. This interpretation was to be tested to determine whether there is a detectable difference in glucose production by the liver of those individuals who have a total absence of glucose 6 phosphatase in contrast with those with a partial defect. Similarly, is there a difference in patients with translocase I defect compared with glucose 6 phosphate defect? Is there a difference in liver glucose production by patients with the translocase I defect who have milder manifestations when compared with the more severely affected?