The metabolic disturbances in
glucose-6-phosphatase deficiency (
von Gierke's disease) are the consequence of
hypoglycemia, occurring mostly during the night. Continuous provision of
glucose is the aim of every recently introduced treatment procedure. We studied the influence of
continuous ambulatory peritoneal dialysis (
CAPD) on the metabolic disturbances in a 42-year-old female patient with
von Gierke's disease and
end-stage renal disease. During six months of
CAPD, there were no dialysis-related complications. The
metabolic acidosis didn't worsen: arterial
bicarbonate and
lactate were not changed. Mean glycemia was 118.6 +/- 14.4 mg%. Total
lipemia,
cholesterol and
triglycerides were not different from those before
CAPD, despite the fact that all hypolipidaemic drugs were stopped. Three different exchange procedures were compared during the night: no dialysis, one exchange with a 2 L
solution without
buffer containing
glucose 15 g/L and containing
glucose 42.5 g/L. The results show that the 4.25%
glucose solution prevents hypoglycaemia, and diminishes the increase in
lactate and
pyruvate concentration. Intraperitoneal
glucose normalizes the plasma
free fatty acid concentration. A very important result is the disappearance of hypo-insulinism. We conclude that, from a clinical point of view,
CAPD is a well-tolerated treatment in
von Gierke's disease. The limited results provide some evidence that the use of a 4.25%
glucose solution as an overnight exchange, instead of the usual 1.5%
solution, can prevent at least partly the glycogenolysis and consequently the metabolic disturbances of
von Gierke's disease.