Effect of ramipril in a patient with glycogen storage disease type I and nephrotic-range proteinuria.

We studied the effect of ramipril on urinary protein excretion and arterial pressure in a 27-year-old patient with GSD Ia and heavy proteinuria (2-3 g /24 h). Ramipril therapy resulted in an important reduction of proteinuria (0.3-0.5 g/24 h): no changes were observed in arterial pressure and renal function during the 12-month follow-up. We conclude that treatment with ramipril can be employed effectively and safely in GSD Ia patients with nephrotic-range proteinuria.