Hereditary fructose intolerance (HFI) is a metabolic disease caused by the absence of an enzyme called aldolase B. In people with HFI, ingestion of fructose (fruit sugar) and sucrose (cane or beet sugar, table sugar) causes severe hypoglycemia (low blood sugar) and the build up of dangerous substances in the liver.

Hereditary fructose intolerance (HFI) is an inborn error of fructose metabolism caused by a deficiency of the enzyme aldolase B. [1] Individuals affected with HFI are asymptomatic until they ingest fructose, sucrose, or sorbitol.

Jul 8, 2007 · 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.

May 29, 2023 · Hereditary fructose intolerance is an autosomal recessive disorder where patients lack the enzyme to break down fructose-1-phosphate. This disease usually presents during the weaning period with nausea, vomiting, jaundice, and hypoglycemia. Early recognition and prompt institution of dietary measures to restrict fructose are essential.

Dec 17, 2015 · Following dietary exposure to fructose, sucrose, or sorbitol, untreated hereditary fructose intolerance (HFI) is characterized by metabolic disturbances (hypoglycemia, lactic acidemia, hypophosphatemia, hyperuricemia, hypermagnesemia, hyperalaninemia) and clinical findings (nausea, vomiting, and abdominal distress; chronic growth restriction / f...

Hereditary fructose intolerance is a condition that affects a person's ability to digest the sugar fructose. Explore symptoms, inheritance, genetics of this condition.

Hereditary fructose intolerance (HFI) is an autosomal recessive disorder of fructose metabolism (see this term), resulting from a deficiency of hepatic fructose-1-phosphate aldolase activity and leading to gastrointestinal disorders and postprandial hypoglycemia following fructose ingestion.

Hereditary fructose intolerance (HFI) is a rare autosomal recessive inherited disorder that occurs due to the mutation of enzyme aldolase B located on chromosome 9q22.3. A fructose load leads to the rapid accumulation of fructose 1-phosphate and manifests with its downstream effects.

Hereditary fructose intolerance (HFI) is a rare inborn disease characterized by a deficiency in aldolase B, which catalyzes the cleavage of fructose 1,6-bisphosphate and fructose 1-phosphate (Fru 1P) to triose molecules.

HFI is a congenital error in fructose metabolism, an autosomal recessive metabolic disease resulting from a deficiency in aldolase B gene function . First described in 1956, HFI is characterized by a “functional deficiency of fructose-1-aldolase activity” [ 4 ].