NCT02979106

Metabolic Consequences of Heterozygous Hereditary Fructose Intolerance

Are Heterozygous Carriers for Hereditary Fructose Intolerance Predisposed to Metabolic Disturbances When Exposed to Fructose?

• Status: Completed
• Phase: N/A
• Study type: Interventional
• Enrollment: 18 (actual)
• Sponsor: University of Lausanne · Academic / Other
• Sex: All
• Age: 18 Years – 65 Years
• Healthy volunteers: Accepted

Summary

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.

Conditions

• Hereditary Fructose Intolerance
• Fructose Metabolism, Inborn Errors
• Glucose Metabolism Disorders

Interventions

Type	Name	Description
OTHER	Test meal	Assessment of postprandial responses to a mixed meal containing fructose in carriers of one mutated ALDOB allele.

Timeline

• Start date: 2015-01-01
• Primary completion: 2016-01-01
• Completion: 2016-11-01
• First posted: 2016-12-01
• Last updated: 2019-07-17