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Study of A Disintegrin and Metalloproteinase with ThromboSpondin Type 1 Repeats 13 (ADAMTS 13) in Children with Idiopathic Nephrotic Syndrome

Background: Nephrotic syndrome (NS) is a well-known risk factor for arterial or venous thromboembolism (TE). There is higher risk of TE in steroid-resistant NS (SRNS) than in steroid-sensitive NS (SSNS). Objective: The aim of this study was to investigate serum level of von Willbrand factor cleaving protease activity which is known as ADAMTS in children with idiopathic nephrotic Syndrome(INS) and its relation to clinical and laboratory parameters.

Patients and methods: This study was conducted on 120 children with INS including 40 SSNS, 40 SRNS, 40 healthy controls. All subjects are investigated by CBC, 24 hours collected urine analysis for urine volume, urinary proteins, total Serum protein and serum albumin,total serum cholesterol,prothrombi time (PT), partial thromboblastin time(PTT) and Serum ADAMTS 13 activity.

Results: There was highly significant decrease in serum ADAMTS 13 activity in SSNS and SRNS groups when compared to control group while there was no significant difference in serum ADAMTS 13 activity between SSNS and SRNS groups.

Conclusion: Serum ADAMTS 13 activity is a biomarker for endothelial dysfunction and hypercoagulable state. The decreased ADAMTS13 activity in different extent of nephrotic patients (SSNS and SRNS) may be one of the pathogenesis of thrombosis as a common complication of NS.

Recommendation: Regular follow-up of nephrotic patients and estimation of serum ADAMTS 13 level as its decresed level is a risk factors of thrombosis.


Mohamed Abdelaziz El- Gamasy, Maher Ahmed Abdelhafez, Mohamed Mahmoud Abdelmageed, Ashraf Sayed Kame

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