Hereditary fructose intolerance (HFI) is the result of a deficiency of the fructose-1-phosphate aldolase (ALDOB) enzyme found in the liver, kidneys and intestine (1). ALDOB is responsible for the conversion of fructose-1-phosphate to glyceraldehyde and dihydroxyacetone-phosphate (1,2). HFI is a rare disease of autosomal recessive inheritance associated with different mutations in the ALDOB gene, located on chromosome 9q31.1 (1-3).

 

Sucrose and sorbitol are both metabolized