Abstract
Three Patients with glycogen storage disease type I were studied by 31phosphorous magnetic resonance spectroscopy. During fasting state, phosphomonoester (PME) peak was higher and inorganic phosphate (Pi) peak was lower in GSD livers than those of control. After intravenous glucose loading(0.5g/kg), PME peak decreased and Pi peak increased in the patient' livers. From chemical shift measurement, the main component of PME peak in GSD liver was thought to be sugar phosphate, probably glucose-6-phosphabe. One of the patients had a hepatic tumor, which had been observed for 6 years without progression. The spectrum of the hepatic tumor had relatively weak signal. The PME peak of the tumor was high compared with ATP peaks. The PME peak was depressed by glucose loading. From above data, we speculate that the hepatic tumor of GSD patient has an accumulation of sugar phosphate, glucose-6-phosphate, by the same enzyme deficiency.
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