Metabolic Consequences of Heterozygous Hereditary Fructose Intolerance

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Rėmėjai

University of Lausanne

KLINIKINIS TYRIMAS: NCT02979106

BioSeek: nct02979106

Raktažodžiai

Santrauka

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.

Datos

Paskutinį kartą patikrinta:	06/30/2019
Pirmasis pateikimas:	11/13/2016
Numatytas registravimas pateiktas:	11/27/2016
Pirmas paskelbtas:	11/30/2016
Paskutinis atnaujinimas pateiktas:	07/14/2019
Paskutinis atnaujinimas paskelbtas:	07/16/2019
Faktinė studijų pradžios data:	12/31/2014
Numatoma pirminio užbaigimo data:	12/31/2015
Numatoma studijų užbaigimo data:	10/31/2016

Būklė ar liga

Hereditary Fructose Intolerance

Fructose Metabolism, Inborn Errors

Glucose Metabolism Disorders

Intervencija / gydymas

Other: oral fructose load

Fazė

Rankų grupės

Ranka	Intervencija / gydymas
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.

Tinkamumo kriterijai

Amžius, tinkami studijuoti	18 Years Į 18 Years
Tinkamos studijoms lytys	All
Priima sveikus savanorius	Taip
Kriterijai	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

Rezultatas

Pirminės rezultatų priemonės

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

Antrinės rezultatų priemonės

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

Raktažodžiai

karbamidas

triglyceride

uric acid

hyperuricemia

insulin resistance

hipoglikemija

hyperlipidemias

dyslipidemias

acidozė

fructose intolerance

carbohydrate

fructose

creatinine