n South African Journal of Child Health - Tissue factor pathway inhibitor in paediatric patients with nephrotic syndrome




Tissue factor pathway inhibitor (TFPI) 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.

To evaluate plasma TFPI levels in paediatric patients with nephrotic syndrome (NS) and its correlation with disease activity.
Fifteen nephrotic patients in relapse (proteinuria >40 mg/m2/h, hypo-albuminaemia and oedema) before initiating steroid therapy (group I) and another 15 nephrotic patients in remission after withdrawal of steroid therapy (group II) were compared with 15 age- and sex-matched healthy children (group III). Besides clinical evaluation and routine laboratory investigations of NS, tissue factor pathway inhibitor levels in plasma were measured by enzyme-linked immunosorbent assay (ELISA).
The plasma TFPI level was higher in nephrotic patients during relapse (group I) and during remission (group II) (mean 102.53 (standard deviation (SD) 14.23) and 82.93 (SD 3.83) ng/ml, respectively) compared with that in the control group (62.40 (SD 7.53) ng/ml) (<0.0001). In children with NS the plasma TFPI level was higher during relapse (group I) compared with the level in remission (group II) (<0.0001). There was a negative correlation between the plasma TFPI level and total protein and serum albumin, and a positive correlation between the plasma TFPI level and the urinary protein/creatinine ratio (<0.05).
NS was associated with increased level of plasma TFPI in comparison with the control group, and the increase was more apparent in patients with active disease.


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