Study of hereditary fructose intolerance by use of 31P magnetic resonance spectroscopy.
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.
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
D005636:Fructosephosphates; C032284:fructose-1-phosphate; D014527:Uric Acid; D010758:Phosphorus; D005632:Fructose; D005634:Fructose-Bisphosphate Aldolase