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 ?
Simple, rapid nonradioactive method to detect the three most prevalent hereditary fructose intolerance mutations.
Obviously, this "condition" is essentially different from the inborn error of metabolism, hereditary fructose intolerance.
It should be preceded by careful evaluation of the patient's history with reference to the possibility of hereditary fructose intolerance.
The report by Wenzel and colleagues highlights the risks inherent in fructose tolerance testing and the challenges accompanying evaluation for hereditary fructose intolerance (HFI) (1).
Molecular analysis of aldolase 8 genes in hereditary fructose intolerance.
Iatrogenic deaths in hereditary fructose intolerance.
DNA diagnosis of fatal fructose intolerance from archival tissue.
Molecular analysis of common aldolase 8 alleles for hereditary fructose intolerance in North Americans.

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