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Completed

NCT Number: NCT02979106

Metabolic Consequences of Heterozygous Hereditary Fructose Intolerance

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.

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Key information

Conditions

Hereditary Fructose Intolerance Carbohydrate Metabolism, Inborn Errors Congenital, Hereditary, and Neonatal Diseases and Abnormalities Fructose Intolerance Fructose Metabolism, Inborn Errors Genetic Diseases, Inborn Glucose Metabolism Disorders Metabolic Diseases Metabolism, Inborn Errors Nutritional and Metabolic Diseases

Age range

18 year–65 year

Sex eligibility

All sexes

Study type

Interventional

Phase

Not applicable

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • 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

Exclusion criteria

  • 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)
  • Drugs
  • Pregnancy

Treatment and study plan

Test meal

Other

Assessment of postprandial responses to a mixed meal containing fructose in carriers of one mutated ALDOB allele.

Primary outcomes

  1. Plasma glucose kinetics

    Time frame: -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

Secondary outcomes

  1. Energy expenditure rate

    Time frame: 120 min before ingestion of a test meal, and every 30 min until 360 min after ingestion of a test meal

    Energy expenditure is measured by indirect calorimetry in fasted and fed conditions

  2. Glucose oxidation rate

    Time frame: 120 min before ingestion of a test meal, and every 30 min until 360 min after ingestion of a test meal

    glucose oxidation is measured by indirect calorimetry in fasted and fed conditions

  3. Plasma glucose concentration

    Time frame: -120 min before ingestion of a test meal, and every 30 min until 360 min after ingestion of a test meal

    plasma glucose concentration measured by glucose oxidase

  4. plasma insulin concentration

    Time frame: -120 min before ingestion of a test meal, and every 30 min until 360 min after ingestion of a test meal

    Plasma insulin concentration measured by ELISA

  5. Fructose oxidation

    Time frame: Every 30 min until 360 min after ingestion of a test meal

    Fructose oxidation is measured from 13CO2 production

Sponsors and collaborators

Lead sponsor

University of Lausanne

Other

Registry information

Official study title

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

Important dates

Study start
2015
Primary completion
2016
Study completion
2016
First posted
Dec 1, 2016
Registry last updated
Jul 17, 2019

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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