Fructose Intolerance, Hereditary National Organization for Rare
Disorders, Inc. It is possible that the main title of the report
Fructose Intolerance, Hereditary is not the name you expected.
Please check the synonyms listing to find the alternate name(s)
and disorder subdivision(s) covered by this report. There are
three inherited disorders of fructose metabolism that are
recognized and characterized. Essential fructosuria, is a mild
disorder not requiring treatment, while Hereditary fructose
intolerance (HFI) and Hereditary fructose-1,6-biphosphatase
deficiency (HFBP) are treatable and controllable but must be
taken seriously. Hereditary Fructose Intolerance (HFI) is an
inherited inability to digest fructose (fruit sugar) or its
precursors (sugar, sorbitol and brown sugar). This is due to a
deficiency of activity of the enzyme fructose-1-phosphate
aldolase, resulting in an accumulation of fructose-1-phosphate
in the liver, kidney, and small intestine. Fructose is a
naturally occurring sugar that is used as