GLYCOGEN STORAGE DISEASE AND THE EFFECT OF DICHLORACETATE IN TYPE IA GLYCOGENESIS

对施用改性玉米淀粉的血糖反应和 支链淀粉-与未修饰的形式相比， 对糖原修饰形式的血糖反应较小。 积累的证据令人信服地指出， 肾功能时，血糖水平维持与玉米 淀粉.如果长期观察支持这种印象，玉米淀粉 治疗将对I型糖尿病的预后产生显著的长期影响。 糖原累积病（GSD）。 本研究旨在确定葡萄糖产生的速度， I型糖原累积症患者的肝脏。III型GSD患者 作为对照GSD受试者进行研究。最新数据的计算 尚未完成。 型糖尿病患者口服二氯醋酸钠的药代动力学及疗效观察 3例患者出现Ia糖原增多症。

The glycemic response to the administration of modified corn starch and amylopectin-compared with the unmodified form has consistently shown a lesser glycemic response to the modified forms of glycogen. The accumulated evidence convincingly points to a beneficial effect on kidney function when the blood glucose level is maintained with corn starch. If long-term observations support this impression, corn starch therapy will have a significant long-term impact on prognosis of type I glycogen storage disease (GSD). This study is designed to determine the rate of glucose production by the liver in individuals with type I glycogenosis. Patients with type Ill GSD were studied as control GSD subjects. The calculations of the latest data is not yet complete. The pharmacokinetics and the effect of dichloracetate in patients with type Ia glycogenosis were studied in 3 patients.