Glycogen storage disease: effects of glucose infusions on [15N]glycine kinetics and nitrogen metabolism.

The effects of intragastric glucose infusions on [15N]glycine kinetics and whole body protein turnover were investigated in four children with type I and two children with type III glycogen storage disease. Either fasting or the administration of insufficient glucose was associated with a diminution in the glycine pool size relative to values observed when patients received adequate glucose. The cause of the smaller pool size was an increased fractional glycine turnover. Simultaneous determination of whole body protein turnover, using a stochastic model based on [15N]urea excretion, showed higher rates of protein synthesis, nitrogen flux, net tissue protein retention, and reutilization of amino acid nitrogen derived from protein catabolism, in patients receiving sufficient exogenous glucose. Depletion of amino acid pools, presumably because of intensive utilization of these gluconeogenic precursors when inadequate glucose is administered, was associated with a lower rate of whole body protein synthesis.