METABOLISM IN CHILDREN WITH GLYCOGEN STORAGE DISEASE, TYPE I

本研究旨在确定葡萄糖产生的速率， 葡萄糖6磷酸酶缺乏、葡萄糖6 磷酸酶和易位酶I缺陷以及III型糖原累积病。 有报道称，I型糖原病患者的肝脏 产生一些葡萄糖 将对该解释进行测试，以确定 肝脏产生的葡萄糖是否存在可检测的差异 对于那些完全缺乏葡萄糖6磷酸酶的人来说， 与部分缺陷的人形成对比。 同样，是否有一个 与葡萄糖6相比，易位酶I缺陷患者的差异 磷酸盐缺陷？ 是否有不同的肝脏葡萄糖的生产， 具有移位酶I缺陷的患者，当 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?