A disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 in children with end-stage renal disease on regular hemodialysis

Abstract

Background: In chronic kidney disease, systemic inflammation may contribute to the endothelial dysfunction and accelerated thrombosis. Vascular access thromboembolism is the most common cardiovascular complication of children with end-stage renal disease (ESRD). Recently, a disintegrate-like and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS13) is suspected to be involved as a specific von Willebrand factor cleaving protease. Objectives: To evaluate serum ADAMTS13 level in children with ESRD on regular hemodialysis (HD) and its correlation to thrombotic episode in HD patients. Materials and Methods: The present study was carried out on forty children with ESRD on regular HD in Pediatric Department of Tanta and Aswan University hospitals and forty healthy age- and sex-matched children were serving as controls. All patients and controls were subjected to thorough history taking, especially history concerning vascular access thrombosis, clinical examination including anthropometric measurements, and routine laboratory assessment measuring complete blood count (CBC), blood urea, serum creatinine, parathormone, prothrombin time, partial thromboplastin time, bleeding time, clotting time, blood electrolytes, and urine analysis. Laboratory investigations also included serum ADAMTS 13 level in this study. Results: There was a positive history of thrombi formation in patients more than controls, especially in the vascular access, and there was a significant decrease in ADAMTS13 level in patients when compared to controls. Conclusions: Diminished serum ADAMTS13 level is an early biomarker for hypercoagulability and thrombotic tendency. Children with ESRD under regular HD have lower levels of serum ADAMTS 13 than controls which increases the risk for thrombosis.