EFFECTIVE RENAL PLASMA-FLOW IN PATIENTS WITH GLYCOGEN-STORAGE-DISEASETYPE-I

To evaluate effective renal plasma flow (ERPF) we performed renal scin tigraphies with Tc-99m-Mercaptoacetyl-triglycine (MAG3) in nine patien ts with glycogen storage disease I (GSD I) (age: 16 +/- 7 years). Two patients presented with proteinuria, none showed hyperaminoaciduria, d isturbed tubular reabsorption of phosphate or hypertension. Tc-99m-MAG 3 clearance values were elevated in eight out of nine patients (865 +/ - 233 ml/min/1.73 m2 body surface area) and exceeded the age-dependent mean values by 21%-145 %. ERPF values in patients with poor metabolic control were higher than in patients with long-term good metabolic co ntrol (988 +/- 186 vs. 619 +/- 55 ml/min/1.73 M2 ; P < 0.05). We concl ude that enhanced ERPF is a common finding in GSD I patients, which pr eceeds clinically overt nephropathy. Renal scintigraphy with Tc-99m MA G3 is a suitable method for the early detection and monitoring of kidn ey dysfunction in GSD I.