Advertisement

Topics

Metabolic Consequences of Heterozygous Hereditary Fructose Intolerance

2016-12-02 16:38:22 | BioPortfolio

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.

Study Design

Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Basic Science

Conditions

Hereditary Fructose Intolerance

Intervention

Test meal

Status

Completed

Source

University of Lausanne

Results (where available)

View Results

Links

Published on BioPortfolio: 2016-12-02T16:38:22-0500

Clinical Trials [865 Associated Clinical Trials listed on BioPortfolio]

Fructose Supplementation in Carriers for Hereditary Fructose Intolerance

This study aimed to examine metabolic response to a short-term fructose enriched diet in carriers for hereditary fructose intolerance compared to controls. Effects of fructose coffees will...

Effect of Fructose on Colonic Microflora

Fructose is the principal carbohydrate in fruits and is added to many food products. This 5 carbon sugar is associated with a number of ill effects, like promotion of high triglycerides,pr...

Effect of Dietary Fat When Eaten With Fructose Versus Glucose

This study is designed to test the effects on liver fat of varying fat intake in the presence of fructose or glucose. We hypothesize that higher dietary fat when eaten with fructose as com...

Fructose Breath-testing in Irritable Bowel Syndrome (IBS)

Background: Breath testing for food intolerances is becoming routine in patients with functional gastrointestinal disorders (FGID). Both FGID and saccharide intolerances (FODMAPs: fermenta...

Monitoring of Hepatic Fat Metabolism Using Magnetic Resonance Methods

The first primary research objective of the study is to determine whether high-fat load (150 g of fat) induces increase in hepatic fat content (HFC) three and six hours after meal in insul...

PubMed Articles [6066 Associated PubMed Articles listed on BioPortfolio]

The Predictive Value of the Hydrogen Breath Test in the Diagnosis of Fructose Malabsorption.

Fructose malabsorption is commonly diagnosed by the hydrogen fructose (H2) breath test. However, the mechanisms behind fructose malabsorption in humans are not well understood and the clinical relevan...

Repercussions of low fructose-drinking water in male rats.

Fructose consumption has increased worldwide, and it has been associated with the development of metabolic diseases such as insulin resistance (IR) and steatosis. The aim was to evaluate if lower fruc...

Effects of fructose-containing sweeteners on fructose intestinal, hepatic, and oral bioavailability in dual-catheterized rats.

Fructose is commonplace in Western diets and is consumed primarily through added sugars as sucrose or high fructose corn syrup. High consumption of fructose has been linked to the development of metab...

Feeding intolerance alters the gut microbiota of preterm infants.

Feeding intolerance (FI) is a common disease in preterm infants, often causing a delay in individual development. Gut microbiota play an important role in nutrient absorption and metabolism of preterm...

Differentiation of functional gastrointestinal disorders from healthy volunteers by lactulose hydrogen breath test and test meal.

Functional dyspepsia (FD) is a common disorder of gut-brain interaction with incompletely understood pathophysiology. Consequently, heterogeneous expert opinions on diagnostic tests and assessment of ...

Medical and Biotech [MESH] Definitions

Inherited abnormalities of fructose metabolism, which include three known autosomal recessive types: hepatic fructokinase deficiency (essential fructosuria), hereditary fructose intolerance, and hereditary fructose-1,6-diphosphatase deficiency. Essential fructosuria is a benign asymptomatic metabolic disorder caused by deficiency in fructokinase, leading to decreased conversion of fructose to fructose-1-phosphate and alimentary hyperfructosemia, but with no clinical dysfunction; may produce a false-positive diabetes test.

An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.

An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Passage of food (sometimes in the form of a test meal) through the gastrointestinal tract as measured in minutes or hours. The rate of passage through the intestine is an indicator of small bowel function.

An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

More From BioPortfolio on "Metabolic Consequences of Heterozygous Hereditary Fructose Intolerance"

Advertisement
Quick Search
Advertisement
Advertisement

 

Relevant Topics

Gout
According to the National Arthritis Data Workgroup, an estimated 6 million people in the United States report having experienced gout at some point in their lives. In fact, gout is the most common form of inflammatory arthritis in men over the age of 40....

Renal disease
Chronic kidney disease (CKD), also known as chronic renal disease, is a progressive loss in renal function over a period of months or years. The symptoms of worsening kidney function are non-specific, and might include feeling generally unwell and experi...


Searches Linking to this Trial