Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition 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. Disease data Classification Disease Synonyms Hereditary fructose-1-phosphate aldolase deficiency Dziedziczna fruktozemia Dziedziczny Niedobór aldolazy fruktozo-1-fosforanu Hereditary fructosemia ORPHA code 469 OMIM code 229600 ICD10 code E74.1 ICD11 code 5C51.50 *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl