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Hereditary fructose intolerance

Synonym(s) Hereditary fructose-1-phosphate aldolase deficiency
Hereditary fructosemia
Prevalence 1-9 / 100 000
Inheritance Autosomal recessive
Age of onset Infancy
  • E74.1
  • D005633
  • 10019878


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

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