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BACKGROUND: Tissue factor pathway inhibitor is an endogenous protease inhibitor that regulates the initiation of the extrinsic coagulation pathway by producing factor Xa-mediated feedback inhibition of the tissue factor/factor VIIa (TF/VIIA) catalytic complex. OBJECTIVES: To evaluate plasma TFPI levels in paediatric patients with nephrotic syndrome and its correlation with disease activity. SUBJECTS AND METHODS: Fifteen nephrotic patients in relapse (proteinuria>40mg/m2/hour, hypoalbuminemia, and edema) before initiating steroid therapy (Group I), and another15 nephrotic patients in remission after withdrawal of steroid therapy (Group II) were compared to 15 age- and sex-matched healthy children (Group III). Besides clinical evaluation and routine laboratory investigations of nephrotic syndrome, tissue factor pathway inhibitor levels in plasma were measured by ELISA. RESULTS: Plasma TFPI level was higher in nephrotic patients during relapse (Group I) and during remission (Group II) [102.53±14.23 and 82.93±3.83ng/ml respectively] compared to that in the control group (62.40 ± 7.53 ng/ml) (p< 0.0001). In children with nephrotic syndrome Plasma TFPI level was higher during relapse (Group I) compared to that in remission (Group II) (p< 0.0001). There was a negative correlation between plasma TFPI level and total protein and serum albumin, and there was a positive correlation between plasma TFPI level and urine protein /creatinine ratio with a statistically significant difference (p< 0.05). CONCLUSION: Nephrotic syndrome was associated with increased level of plasma tissue factor pathway inhibitor in comparison to control group and the increase was more apparent in patients with active disease.

Tissue factor pathway inhibitor in paediatric patients with nephrotic syndrome.