Glucose and leucine kinetics in idiopathic ketotic hypoglycaemia
Glucose and leucine kinetics in idiopathic ketotic hypoglycaemia
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DOI：
10.1136/adc.2005.089425
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发表时间：
2006-06-01
影响因子：
5.2
通讯作者：
Leonard, J. V.
中科院分区：
文献类型：
--
作者：
Bodamer, O. A.;Hussein, K.;Leonard, J. V.
目的：研究酮症低血糖（KH）患儿的葡萄糖和亮氨酸动力学与代谢和内分泌检查的关系，以阐明潜在的病理生理学。方法：采用稳定同位素示踪剂对9名儿童进行前瞻性干预研究（平均年龄4.23岁，范围0.9 - 9.8岁; 7名男性），KH组和11名对照组（平均年龄4.57岁，范围0.16 - 12.3岁; 4名男性）。结果：与非KH组的受试者相比，KH组的血浆胰岛素水平显著降低。KH组血浆酮体水平显著高于非KH组。KH受试者的基础代谢率显著更高（45.48 +/- 7.41 vs 31.81 +/- 6.72 kcal/kg/d），但两组的呼吸抑制率相似（KH vs非KH，0.84 +/- 0.05 vs 0.8 +/- 0.04）。患有KH的儿童的亮氨酸氧化率显著较低（12.25 +/- 6.25 vs 31.96 +/- 8.59 μ mol/kg/h）。肝葡萄糖的生产率也显着降低KH（3.84 +/- 0.46比6.6 +/- 0.59 mg/kg/min）。结论：KH是由未能维持肝葡萄糖的生产，而不是增加葡萄糖氧化率。能量需求显著增加，而亮氨酸氧化减少。
Aims: To investigate glucose and leucine kinetics in association with metabolic and endocrine investigations in children with ketotic hypoglycaemia (KH) in order to elucidate the underlying pathophysiology.Methods: Prospective interventional study using stable isotope tracer in nine children (mean age 4.23 years, range 0.9 - 9.8 years; seven males) with KH and 11 controls (mean age 4.57 years, range 0.16 - 12.3 years; four males).Results: Plasma insulin levels were significantly lower in KH compared to subjects in the non-KH group. Plasma ketone body levels were significantly higher in KH than in non-KH. Basal metabolic rate was significantly higher in subjects with KH (45.48 +/- 7.41 v 31.81 +/- 6.72 kcal/kg/day) but the respiratory quotients were similar in both groups (KH v non-KH, 0.84 +/- 0.05 v 0.8 +/- 0.04). Leucine oxidation rates were significantly lower in children with KH (12.25 +/- 6.25 v 31.96 +/- 8.59 mu mol/kg/h). Hepatic glucose production rates were also significantly lower in KH (3.84 +/- 0.46 v 6.6 +/- 0.59 mg/kg/min).Conclusions: KH is caused by a failure to sustain hepatic glucose production rather than by increased glucose oxidation rates. Energy demand is significantly increased, whereas leucine oxidation is reduced.