fructose intolerance


Also found in: Dictionary, Thesaurus, Encyclopedia.
Related to fructose intolerance: lactose intolerance, Hereditary fructose intolerance

fructose intolerance

Inability to metabolize the carbohydrate fructose due to a hereditary absence or deficiency of the enzyme 1,6-biphosphate aldolase B. Clinical signs develop early in life. They include hypoglycemia, jaundice, hepatomegaly, vomiting, lethargy, irritability, and convulsions. Fructose can be identified in the urine. The fructose tolerance test should not be used because it can induce irreversible coma.

Treatment

Acute attacks are treated by glucose administration. For long-term therapy, all foods containing fructose (present in sweet fruits and sugar cane) and sucrose and sorbitol (the latter used as a sweetening agent in foods and drugs) must be eliminated from the diet.

References in periodicals archive ?
Cause Assessment method Syphilis Quantitative nontreponemal serologic test Toxoplasmosis Serology (IgG and IgM) Rubella Serology (IgG and IgM) Cytomegalovirus Serology (IgG and IgM) and urinary PCR Herpes simplex virus Serology (IgG and IgM) Parvovirus B19 PCR Infection/sepsis Blood culture, urine culture, and chest X-ray Hepatitis viruses A, B, and C Serology (IgG and IgM) Tyrosinemia Urinary succinylacetone Galactosemia Galactose-1-phosphate uridyltransferase Hereditary fructose intolerance Transferrin isoelectric focusing Niemann-Pick type C disease Filipin test Gaucher disease Leukocyte glucocerebrosidase activity Alpha-1-antitrypsin deficiency Detection of PiZ allele PCR = polymerase chain reaction.
Symptoms were reported by a majority of those with positive breath tests for fructose intolerance. All patients were asked to go on a fructose-restricted diet.
Treatment of fructose intolerance begins with a shift to a low-fructose diet.
The only way to reduce the pain, bloating, heartburn and diarrhoea associated with fructose intolerance is to completely eliminate fructose from your diet.
* If hereditary fructose intolerance cannot be ruled out by nutritional history, genetic testing for HFI is advisable (e.g., sequence analysis of exons 2-9 of the ALDOB gene), which leads to a definitive diagnosis in more than 90% of all HFI cases.
Molecular analysis of the aldolase B gene in patients with hereditary fructose intolerance from Spain.
1A are the IEF transferrin profiles for alcohol abuse (lane 6, double bands attributable to a polymorphism in the protein part of transferrin), for galactosemia (lane 5), and for fructose intolerance (lane 4).
The result of the present study indicate that more than one-third of patients diagnosed with irritable bowel syndrome have fructose intolerance and that symptoms improve in these patients on f fructose-restricted diet.
Fructose intolerance in IBS and utility of fructose-restricted diet.
Hereditary fructose intolerance (HFI) is an autosomal recessive disorder, the true incidence of which is not known but may be estimated as high as 1 of 20 000 (1, 2).
Molecular basis of hereditary fructose intolerance: mutations and polymorphisms in the human aldolase 8 gene.

Full browser ?