title: Study of hereditary fructose intolerance by use of 31P magnetic resonance spectroscopy.
abstract: The effect of fructose on liver metabolism in patients with hereditary fructose intolerance (HFI) and in heterozygotes for HFI was studied by 31P magnetic resonance spectroscopy (31P-MRS). In patients with HFI (n = 5) ingestion of small amounts of fructose was followed by an increase in sugar phosphates and decrease in inorganic phosphate (Pi) in the liver that could be detected by 31P-MRS. 31P-MRS could be used to diagnose fructose intolerance and to monitor the patients' compliance with a fructose-restricted diet. In heterozygotes (n = 8) 50 g fructose given orally led to accumulation of sugar phosphates and depletion of Pi in the liver. Fructose also induced a larger increase in plasma urate in heterozygotes than in control subjects. The effect of fructose on liver Pi and plasma urate was most pronounced in heterozygotes with gout (n = 3). Heterozygosity for HFI may predispose to hyperuricaemia.
mesh_terms: D002239:Carbohydrate Metabolism, Inborn Errors / Q000378:metabolism*; D005260:Female; D005632:Fructose / Q000494:pharmacology; D005633:Fructose Intolerance / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism*; D005634:Fructose-Bisphosphate Aldolase / Q000032:analysis; D005636:Fructosephosphates / Q000032:analysis; D006073:Gout / Q000378:metabolism; D006579:Heterozygote; D006801:Humans; D008099:Liver / Q000378:metabolism; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D010758:Phosphorus; D014527:Uric Acid / Q000097:blood
chemical_list: D005636:Fructosephosphates; C032284:fructose-1-phosphate; D014527:Uric Acid; D010758:Phosphorus; D005632:Fructose; D005634:Fructose-Bisphosphate Aldolase