[Transient correction of partial congenital factor V deficiency in nephrotic syndrome]

Plasma concentration of several hemostatic proteins may be modified during the acute phase of nephrotic syndrome. The case of such a syndrome in a patient with congenital factor V deficiency is presented. CASE REPORT--A 5 year-old girl with partial congenital factor V deficiency (level: 30%), was admitted for nephrotic syndrome complicating Henoch-Schönlein purpura. Urine protein excretion was 4 g/24 h. Initial plasma 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 methylprednisolone (1 g/1.73 m2) followed by prednisone (2 mg/kg/day). Under this treatment, the plasma concentrations were: protein: 4.5 g/dl; albumin: 2.0 g/dl; factor II: 180%; factor V: 84%; factors VII + X: 120%. Values at the onset of remission were: albumin: 3.4 g/dl; factor V: 49%. CONCLUSION--This observation suggests that hypoalbuminemia may enhance liver synthesis of factor V as known for some other coagulation factors, and transiently correct the hereditary deficiency.