FUEL KINETICS IN DISORDERS OF FASTING HYPOGLYCEMIA

空腹低血糖疾病的燃料动力学

• 批准号: 6116851
• 负责人: CHARLES ALFRED STANLEY
• 金额: $ 2.47万
• 依托单位: CHILDREN'S HOSP OF PHILADELPHIA
• 依托单位国家: 美国
• 财政年份: 1998
• 资助国家: 美国
• 起止时间: 1998-12-01 至 1999-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6116851
• 关键词: aminoacid metabolism; bioenergetics; child (0-11); clinical research; fasting; fatty acid metabolism; gluconeogenesis; glucose metabolism; human subject; hypoglycemia; infant human (0-1 year); metabolism disorder diagnosis; nutrition related tag; stable isotope diagnosis

This study utilizes stable isotope methodologies to quantify the changes in rates of glucose utilization and amino acid oxidation during fasting in children with hypoglycemic disorders. These methods have been applied to evaluate the mechanisms of hypoglycemia in ketotic and hypoketotic forms of hypoglycemia. Preliminary data in children with genetic defects in fatty acid oxidation suggest that they do not have excessive glucose utilization rates, but that, as in normal children, hypoglycemia occurs as a consequence of limited capacity for glucose production. Analysis of changes in amino acid kinetics during fasting may reveal whether this limitation reflects a mismatch between rates of glucose utilization and rates of substrates for glucose production.

这项研究利用稳定的同位素方法来量化变化 禁食期间的葡萄糖利用率和氨基酸氧化速率 降血糖疾病的儿童。这些方法已应用于 评估酮和低血糖形式低血糖的机制 低血糖。患有遗传缺陷儿童的初步数据 脂肪酸氧化表明它们没有过多的葡萄糖 利用率，但与正常儿童一样，低血糖的发生为 葡萄糖产生能力有限的结果。分析 禁食过程中氨基酸动力学的变化可能会揭示 限制反映了葡萄糖利用率和 葡萄糖产生的底物速率。