Hereditary fructose intolerance

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Hereditary fructose intolerance

Synonyms: Hereditary fructose-1-phosphate aldolase deficiency | Hereditary fructosemia

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. HFI is a benign condition when treated but it is life-threatening and potentially fatal if left untreated.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

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Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: // Data version September 2023.

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Hereditary fructose intolerance?

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Advocacy Organizations

Mississippi Metabolics Foundation

Mississippi Metabolics Foundation (MMF) was founded to raise awareness, educate, and provide support to those living or caring for someone with genetic metabolic disorders/inborn errors of metabolism (IEM).

Clinical Trials

For a list of clinical trials in this disease area, please click here.