Are Heterozygous Carriers for Hereditary Fructose Intolerance Predisposed to Metabolic Disturbances When Exposed to Fructose?
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 18
- 主要终点
- Plasma glucose kinetics
研究概览
简要总结
Background: High fructose intake increases blood lactate, triglyceride and uric acid concentrations. Uric acid may contribute to insulin resistance and dyslipidemia in the general population. In patients with hereditary fructose intolerance fructose consumption is associated with acute hypoglycemia, renal tubular acidosis, and hyperuricemia.
Objective: We investigated whether asymptomatic carriers for hereditary fructose intolerance (HFI) would have a higher sensitivity to adverse effects of fructose than the general population.
Design: Eight subjects heterozygous for HFI (hHFI; 4 males, 4 females) and eight controls received for 7 days a low fructose diet and on the eighth day ingested a test meal calculated to provide 25% of basal energy requirement containing labeled fructose (13C fructose 0.35 g/kg), protein (0.21 g/kg) and lipid (0.22 g/kg). Total fructose oxidation, total endogenous glucose production (by 6,6-2H2-glucose dilution), carbohydrate and lipid oxidation, lipids, uric acid, lactate, creatinine, urea and amino acids were monitored for 6 hours.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Basic Science
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 65 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •8 healthy Volunteers (4 male, 4 female) parents of a child with hereditary fructose intolerance with ALDOB with heterozygous mutation of ALDOB gene
- •8 healthy Volunteers (4 male, 4 female), healthy with no mutation of ALDOB gene
排除标准
- •Fasting glycemia > 7.0 mmol/L
- •Fasting total triglycerides > 4.0 mmol/L
- •Chronic renal insufficiency (eGFR ≤ 50 ml/min)
- •Anemia (ferritin < 20 ug/L, hemoglobin < 13.5 ou 12.5 g/dl)
- •Pregnancy
研究组 & 干预措施
oral fructose load
test meal calculated to provide 25% of basal energy requirement containing 13C-labeled fructose (0.35 g/kg), protein (0.21 g/kg) and lipid (0.22 g/kg).
干预措施: Test meal (Other)
结局指标
主要结局
Plasma glucose kinetics
时间窗: -120 min before ingestion of a test meal to 360 min after ingestion of a test meal
Modelling of rate of glucose appearance after administration of a bolus of 6,6-2H2 glucose (bolus, 2 mg/kg and continuous infusion, 0.02 mg/kg/min) will be measured in fasted and fed conditions
次要结局
- plasma insulin concentration(-120 min before ingestion of a test meal, and every 30 min until 360 min after ingestion of a test meal)
- Energy expenditure rate(120 min before ingestion of a test meal, and every 30 min until 360 min after ingestion of a test meal)
- Glucose oxidation rate(120 min before ingestion of a test meal, and every 30 min until 360 min after ingestion of a test meal)
- Plasma glucose concentration(-120 min before ingestion of a test meal, and every 30 min until 360 min after ingestion of a test meal)
- Fructose oxidation(Every 30 min until 360 min after ingestion of a test meal)
研究者
Luc Tappy, MD
Professor
University of Lausanne