Glycogen storage disease type I
Effective renal plasma flow
Springer Online Journal Archives 1860-2000
Abstract To evaluate effective renal plasma flow (ERPF) we performed renal scintigraphies with99mTc-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 with99mTc MAG3 is a suitable method for the early detection and monitoring of kidney dysfunction in GSD I.
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