Fructose intolerance

What is Fructose intolerance?

Hereditary fructose intolerance (HFI) is an inborn error of fructose metabolism caused by a deficiency of the enzyme aldolase B. Individuals affected with HFI are asymptomatic until they ingest fructose, sucrose, or sorbitol. If fructose is ingested, the enzymatic block at aldolase B causes an accumulation of fructose-1-phosphate. This accumulation has downstream effects on gluconeogenesis and regeneration of adenosine triphosphate (ATP). Symptoms of HFI include vomiting, hypoglycemia, jaundice, hemorrhage, hepatomegaly, hyperuricemia and potentially kidney failure. While HFI is not clinically a devastating condition, there are reported deaths in infants and children as a result of the metabolic consequences of HFI. Death in HFI is always associated with problems in diagnosis.

HFI is an autosomal recessive condition caused by mutations in the ALDOB gene, located at 9q22.3. HFI is typically suspected based on dietary history, especially in infants who become symptomatic after breast feeding. This suspicion is typically confirmed by molecular analysis. Treatment of HFI involves strict avoidance of fructose in the diet. Older patients with HFI typically self-select a diet low in fructose, even before being diagnosed.

Questions and answers on "Fructose intolerance"

Hi, I have been suffering from a burning tongue for 2 years. I am an 42 year old female. I was diagnosed through hydrogen breath test with fructose...

doctor1 MD


Welcome to HCM.
Based on the information provided the cause for the burning tongue is most likely to be due to Vitamin deficiency.


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I am 25and weigh 204height 6"1. No real history. I fwwl nauseous all the time but worse when hungry or right after eating. Have seen a GI doc but...

doctor1 MD

Well come to HCM
Thank for asking
I appreciate your concern, in fact underlying cause of vomiting or nausea is matter of concern, if the...

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I have been suffering from severe reflux for 12 months. Drugs have not been successful. I have lost 15 kgs (not originally overweight). I have...

doctor1 MD

Hi, Welcome to health care magic forum.
As you describe, you had a rise in cholesterol, with in 2 months when you was using the egg, regularly....

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