fructose intolerance

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Related to fructose intolerance: lactose intolerance, Hereditary fructose intolerance

fructose intolerance

Etymology: L, fructus, fruit, in + tolerare, to bear
an inherited disorder marked by an absence of enzymes needed to metabolize fructose. Symptoms include sweating, tremors, confusion, digestive distress with vomiting, and failure of infants to grow. The condition is transmitted as an autosomal-recessive trait. Also called 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.


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 ?
Treatment of fructose intolerance begins with a shift to a low-fructose diet.
If hereditary fructose intolerance cannot be ruled out by nutritional history, genetic testing for HFI is advisable (e.
Molecular analysis of the aldolase B gene in patients with hereditary fructose intolerance from Spain.
The spectrum of aldolase B (ALDOB) mutations and the prevalence of hereditary fructose intolerance in Central Europe.
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).
Inhibition of phosphomannose isomerase by fructose-l-phosphate: an explanation for defective N-glycosylation in hereditary fructose intolerance.
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).
Our diagnostic strategy for hereditary fructose intolerance is specific to our population but can be transposed and adapted to other populations in which different mutant alleles of aldolase B contribute to the burden of disease.

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