Fructose Intolerance, Hereditary You are reading a NORD Rare
Disease Report Abstract. NORD’s full collection of reports on
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reports per day to visitors on our Web site. Synonyms of
Fructose Intolerance, Hereditary - Fructose-1-Phosphate Aldolase
Deficiency - No subdivisions found. 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 i