What Is Hereditary Fructose Intolerance (HFI)?

Hereditary fructose intolerance (HFI) is a rare genetic disease in which fructose cannot be properly metabolised in the body. Affected person suffers from a bad abdominal reaction (bloating, diarrhea, generalized malaise) after eating fructose-containing foods. HFI is an autosomal recessive disorder of fructose metabolism, due to a deficiency of fructose-1-phosphate aldolase – an enzyme that converts fructose into glucose in the liver. Incidence of HFI is estimated at 1 / 22-58,000 people (1).

HFI should be differed from fructose malabsorption.


continue reading Rare Genetic Disorders: Hereditary Fructose Intolerance (HFI)