The Lactate Concentration of the Urine, a Parameter for the Adequacy of Dietary Treatment of Patients with Glucose-6-phosphatase Deficiency

Abstract

Gastric drip feeding (GDF) during the night improves the clinical and metabolic abnormalities of patients with glycogen storage disease due to glucose-6-phosphatase (EC 3.1.3.9) deficiency (Greene et al., 1980; Fernandes et al., 1979; Stanley et al., 1981). The normalization of blood glucose by GDF diminishes lactate overproduction by the liver and, therefore, suppresses the tendency for hyperlactacidaemia. Conversely, a (nearly) normal level of blood lactate is considered to be a parameter for adequate glucose supply by GDF (Stanley et al., 1981). In our experience, however, the blood lactate concentration often fluctuates strongly. The lactate concentration of the urine averages these fluctuations and thus reflects lactate overproduction better than incidental blood lactate values do (Fernandes and Blom, 1976).