ramipril and Glycogen-Storage-Disease-Type-I

We studied the effect of ramipril on proteinuria and mild hypertension in a 21-year-old patient affected by glycogen storage disease type I non-A. After few months of therapy we obtained a decrease in total urine protein excretion that later re-increased in spite of the high dose of ACE inhibitor. Even if ACE inhibitors are the only effective therapy for GSD I nephropathy, further studies are requested.

Topics: Adult; Angiotensin-Converting Enzyme Inhibitors; Glucose-6-Phosphatase; Glycogen Storage Disease Type I; Humans; Hypertension, Renal; Kidney Diseases; Male; Proteinuria; Ramipril

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.

Topics: Adult; Angiotensin-Converting Enzyme Inhibitors; Blood Pressure; Glycogen Storage Disease Type I; Humans; Kidney Function Tests; Male; Nephrosis; Proteinuria; Ramipril