Dietary Guidelines for Hereditary Fructose Intolerance (HFI) Food Category Foods Permitted Foods Prohibited; Dairy: Any milk, cheese, eggs: Milk products w/added sugar (sweetened yogurt, fruit yogurt, milkshake, chocolate milk) Meat: Beef, veal, lamb, pork:

Treatment of HFI is currently done through a strict fructose free diet. This involves exclusion of anything containing fructose, sucrose, or sorbitol. Although, implementation can be difficult, below are links to some helpful information.

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.

HFI Treatment. The normal treatment, if you suffer from HFI, is a strict fructose free diet. This involves exclusion of anything containing fructose, sucrose, or sorbitol. Implementation and adherence to this diet is often difficult, but not impossible.

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.

2015年12月17日 · 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 (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.

2023年5月29日 · 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.

2021年2月18日 · Prevention of primary manifestations: Dietary restriction of fructose, sucrose, sucralose, and sorbitol is the cornerstone of HFI treatment. During hospitalizations, special caution is advised to avoid use of fructose-containing intravenous fluids, as well as fructose-containing infant formulas and pharmaceuticals.

2022年7月9日 · 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.