Metabolic Consequences of Heterozygous Hereditary Fructose Intolerance

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Sponsorii

University of Lausanne

STUDIU CLINIC: NCT02979106

BioSeek: nct02979106

Cuvinte cheie

Abstract

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.

Datele

Ultima verificare:	06/30/2019
Primul depus:	11/13/2016
Inscriere estimată trimisă:	11/27/2016
Prima postare:	11/30/2016
Ultima actualizare trimisă:	07/14/2019
Ultima actualizare postată:	07/16/2019
Data actuală de începere a studiului:	12/31/2014
Data estimată de finalizare primară:	12/31/2015
Data estimată de finalizare a studiului:	10/31/2016

Stare sau boală

Hereditary Fructose Intolerance

Fructose Metabolism, Inborn Errors

Glucose Metabolism Disorders

Intervenție / tratament

Other: oral fructose load

Fază

Grupuri de brațe

Braţ	Intervenție / tratament
Experimental: 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).	Other: oral fructose load Assessment of postprandial responses to a mixed meal containing fructose in carriers of one mutated ALDOB allele.

Criterii de eligibilitate

Vârste eligibile pentru studiu	18 Years La 18 Years
Sexe eligibile pentru studiu	All
Acceptă voluntari sănătoși	da
Criterii	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

Rezultat

Măsuri de rezultate primare

1. Plasma glucose kinetics [-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

Măsuri de rezultate secundare

1. Energy expenditure rate [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 [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 [-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 [-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 [Every 30 min until 360 min after ingestion of a test meal]

Fructose oxidation is measured from 13CO2 production

Metabolic Consequences of Heterozygous Hereditary Fructose Intolerance

Cuvinte cheie

uree

triglyceride

uric acid

hyperuricemia

rezistență la insulină

hipoglicemie

hyperlipidemias

dislipidemie

acidoză

fructose intolerance

carbohydrate

fructose

creatinină