Abstract
In patients with glycogen storage disease due to the deficiency of debranching enzyme glycogen is only a limited source of glucose. It is unknown to what extent these patients produce glucose endogenously. We have measured the turnover and endogenous proiction of glucose in patients after fasting by isotope-dilution with 6.6-2H2-glucose. 7 Patients between 1 and 16 yr of age were studied. During the measurement the average glucose concentration in blood was 2.6±0.3mM. The glucose turnover decreased from 6.7±0.5mg/kg min at 1 yr of age to 2.2±0.1 mg/kg min at 16 yr of age. The turnover was 0.73±0.13 of normal. The endogenous production of glucose decreased from 4.9±0.6 mg/kg min at 1 yr of age to 1.6±0.1 at 16 yr of age. In our patients the endogenous glucose production was 0.72±0.07 of the turnover. These data indicate that these patients have a nearly normal glucose turnover and a glucose production too low for the demand.
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Smit, G., Chapman, T., Mulder, I. et al. Glucose metabolism in patients with glycogenosls type III. Pediatr Res 22, 236 (1987). https://doi.org/10.1203/00006450-198708000-00136
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DOI: https://doi.org/10.1203/00006450-198708000-00136