METABOLISM IN CHILDREN WITH GLYCOGEN STORAGE DISEASE, TYPE I

本研究旨在确定葡萄糖的速率 在缺乏葡萄糖的患者中由肝脏产生6 磷酸酶、葡萄糖6磷酸酶缺陷型和 易位酶I以及III型糖原累积症。 有报道 I型糖原增多症患者的肝脏会产生一些 葡萄糖 将对该解释进行测试，以确定 是否存在葡萄糖产生的可检测差异， 那些完全没有葡萄糖的人的肝脏 6磷酸盐与那些部分缺陷。 同样的，在携带转位酶I的患者中是否存在差异 与葡萄糖6磷酸盐缺陷相比？ 有没有 肝葡萄糖生成的差异， 易位酶I缺陷的患者， 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 phosphate 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?