Evaluation of von Willebrand Factor-cleaving Proteinase(vWF-CP) in pediatric patients with primary glomerulonephropathy

Background and Objectives: Nephrotic syndrome (NS) is a well-known risk factor for arterial or venous thromboembolism (TE). There is a higher risk of TE in steroid-resistant nephrotic syndrome (SRNS) than in steroid-sensitive NS (SSNS). The aim of this study was to investigate serum level of von Willebrand factor-cleaving protease activity which is known as vWF-CP in children with idiopathic NS (INS) and its correlation with clinical and laboratory parameters. Patients and Methods: This study was conducted on 120 children with INS, including 40 SSNS, 40 SRNS, and 40 healthy controls. All subjects are investigated by complete blood count, 24 h collected urine analysis for urine volume, urinary proteins, total serum protein and serum albumin, total serum cholesterol, prothrombin time, partial thromboblastin time (PTT), and serum vWF-CP activity. Results: There was a highly significant decrease in serum vWF-CP activity in SSNS and SRNS groups when compared to control group while there was no significant difference in serum vWF-CP activity between SSNS and SRNS groups. Conclusions: Serum vWF-CP activity is a biomarker for endothelial dysfunction and hypercoagulable state. The decreased vWF-CP activity in different extent of nephrotic patients (SSNS and SRNS) may be one of the pathogenesis of thrombosis as a common complication of NS. Regular follow-up of nephrotic patients and estimation of serum vWF-CP level as its decreased level is a risk factor of thrombosis.