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ORIGINAL ARTICLE
Year : 2017  |  Volume : 4  |  Issue : 4  |  Page : 130-135

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


Department of Pediatric, Banha Teaching Hospital, Benha, Qalubia Governate, Egypt

Correspondence Address:
Dr. Hasan Tarek Abd-Allah Ebrahim
Department of Pediatric, Benha Teaching Hospital, Benha, Qalubia Governate
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jina.jina_18_17

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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.


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