Significance of Von Willebrand Factor-Cleaving Protease Activity in Patients with Chronic Kidney Diseases

Background: Chronic kidney disease (CKD) is a major health problem worldwide with increasing incidence and prevalence. ADAMTS13 is a proteolytic enzyme that is responsible for degradation of large multimers of VWF released in the plasma by endothelial cells and platelets. Aim of work: The aim of our study was to assess plasma concentration of von Willebrand factor cleaving protease activity in pediatric patient with chronic kidney disease. Subject and Methods: This case control study was conducted on patients attending Pediatric Nephrology unit and outpatient nephrology Clinic pediatric department from January 2020 to November 2020. They were divided as following: Patient group (Group I): twenty children previously diagnosed with chronic kidney disease on hemodialysis. Patient group (Group II): twenty children previously diagnosed with chronic kidney disease and on conservative therapy. Control group (Group III): twenty children apparently healthy from outpatient clinic. All patients were subjected to history (age, sex, residence, birth order, level of education, socioeconomic status), examination, laboratory investigations and Von Willebrand factor cleaving protease enzyme was assayed. Results: Lower levels of plasma concentration of von Willebrand factor cleaving protease activity was more among hemodialysis than chronic kidney disease on conservative therapy. A statistically significant positive correlation between Von Willebrand factor cleaving protease enzyme e and G.F.R. Conclusion: CKD patients have statistically significant lower levels of plasma concentration of von Willebrand factor cleaving protease activity when compared to healthy controls. Concentration of von Willebrand factor cleaving protease activity was significantly decreased in the studied HD patients with thrombosis compared with those without thrombosis