Abstract
The effect of fructose on liver metabolism was studied by in vivo magnetic resonance spectroscopy in fasting healthy controls and in adults with inherited disorders of fructose metabolism, essential fructosuria (EF), hereditary fructose intolerance (HFI), and fructose-1,6-diphosphatase deficiency (FDD). Novel procedures of spectrometer calibration and spectrum analysis allowed accurate measurements of absolute concentrations of phosphorous compounds in liver. In healthy controls, after fructose infusion (200mg/kg, 20% solution, 2.5min), a fructokinase-mediated, rapid increase of fructose-1-phosphate (F-1-P) from 2.9 to 7.8 mmol per liter of liver was seen, while ATP dropped from 2.7 to 1.8 and inorganic phosphorus (Pi) from 1.4 to 0.3 mmol/l. The subsequent return of F-1-P, mediated by fructaldolase, to its initial concentration was accompagnied by an overshooting rise of Pi up to 2.7 mmol/l. In a patient with EF, concentrations of F-1-P. ATP, and Pi remained unchanged confirming that fructokinase was indeed inactive. In a patient with HFI, initial metabolic changes were as in controls, but baseline concentrations were reestablished only after a tenfold delay indicating weak fructaldolase activity. In a patient with FDD, i.e. the most distal of the three defects, initial metabolic changes occured as in controls at the normal rate and normalization was only slightly delayed.
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Boesiger, P., Buchll, R., Meier, D. et al. 140 METABOLIC CHANGES IN LIVER AFTER INTRAVENOUS FRUCTOSE IN ADULTS WITH DISCORDERS OF FRUCTOSE METABOLISM AND IN HEALTHY CONTROLS, FOLLOWED BY PHOSPHORUS MAGNETIC RESONNANCE SPECTROSCOPY. Pediatr Res 30, 651 (1991). https://doi.org/10.1203/00006450-199112000-00170
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DOI: https://doi.org/10.1203/00006450-199112000-00170