Effective renal plasma flow in patients with glycogen storage disease type I

To evaluate effective renal plasma flow (ERPF) we performed renal scintigraphies with^99mTc-Mercaptoacetyl-triglycine (MAG₃) in ninepatients with glycogen storage disease I (GSD I) (age: 16±7 years). Two patients presented with proteinuria, none showed hyperaminoaciduria, disturbed tubular reabsorption of phosphate or hypertension.^99mTc-MAG₃ clearance values were elevated in eight out of nine patients (865±233 ml/min/1.73 m² body surface area) and exceeded the agedependent mean values by 21%–145%. ERPF values in patients with poor metabolic control were higher than in patients with long-term good metabolic control (988±186 vs. 619±55 ml/min/1.73 m²;P<0.05). We conclude that enhanced ERPF is a common finding in GSD I patients, which preceeds clinically overt nephropathy. Renal scientigraphy with^99mTc MAG₃ is a suitable method for the early detection and monitoring of kidney dysfunction in GSD I.