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A disintegrin and metalloprotenase with thrombospondin type 1 repeats 13 (ADAMTS13) in children with end stage renal disease on regular haemodialysis.

Mohamed Abdelaziz El-Gamasy, Maher Ahmed Abdel Hafez, Mohsen Mustafa Eldeeb, Mohamed Mahmoud Abdelmageed

Background: In chronic kidney disease (CKD), systemic inflammation may contribute to the endothelial dysfunction and accelerated thrombosis. Vascular access thromboembolism is the commonest cardio-vascular complication of children with End Stage Renal Diseae (ESRD). Recently A disintegrin and metalloprotease with thrombospondin type 1 repeats 13 (ADAMTS13) is suspected to be involved as a specific von Willebrand Factor (vWF) Cleaving Protease. Objectives: To evaluate serum ADAMTS13 level in children with ESRD on regular Haemodialysis (HD) and its correlation to thrombotic episode in HD patients. Subjects and methods: The present study was carried out on 40 children with ESRD on regular hemodialysis in Pediatric department of Tanta University hospital and 40 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 and clinical examination including anthropometric measurements, Routine laboratory assessment measuring complete blood picture (CBC) blood urea, serum Creatinin, PTH, PT, PTT, bleeding time, clotting time, blood electrolytes and urine analysis. Laboratory investigations include also 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 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 increase the risk for thrombosis.