TRANSIENT CORRECTION OF A PARTIAL CONGENI TAL FACTOR-V DEFICIENCY IN NEPHROTIC SYNDROME

Plasma concentration of several hemostatic proteins may be modified du ring the acute phase of nephrotic syndrome. The case of such a syndrom e in a patient with congenital factor V deficiency is presented. Case report. - A 5 year-old girl with partial congenital factor V deficienc y (level: 30%), was admitted for nephrotic syndrome complicating Henoc h-Schonlein purpura. Urine protein excretion was 4 g/24 h. Initial pla sma concentrations were: protein: 5.5 g/dl; albumin: 3.3 g/dl; factor II: 85%; factor V: 56%; factors VII + X: 80%. The patient was given me thylprednisolone (1 g/1.73 m2) followed by prednisone (2 mg/kg/day). U nder this treatment, the plasma concentrations were: protein: 4.5 g/dl ; albumin: 2.0 g/dl; factor II: 180%; factor V: 84%; factors VIII + X: 120%. Values at the onset of remission were: albumin: 3,4 g/dl; facto r V: 49%; Conclusion. - This observation suggests that hypoalbuminemia may enhance liver synthesis of factor V as known for some other coagu lation factors, and transiently correct the hereditary deficiency.