Tissue factor pathway inhibitor in childhood nephrotic syndrome
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It is now recognised that the extrinsic tissue factor pathway is the main trigger to the coagulation system in vivo. Its main inhibitor, tissue factor pathway inhibitor (TFPI), has never been studied in childhood nephrotic syndrome. The aim of the study was to monitor the level of TFPI in childhood nephrotic syndrome. One hundred and thirty-nine nephrotic children were classified into the following groups: group 1 (n=25), in relapse and receiving no treatment; group 2 (n=37), in relapse but receiving steroid treatment; group 3 (n= 45), in early remission and on steroids; group 4 (n=24), in established remission and receiving no steroids; group 5 (n=8), steroid-resistant. The controls (n=84) were healthy and age-matched. There was significant elevation of total TFPI levels in groups 1 and 2 and 3; levels were comparable to those of the healthy controls in group 4. The highest levels of total TFPI were recorded in group 5. Like total TFPI, the levels of the free form of TFPI showed a statistically significant increase in groups 1, 2, 3 and 4, when compared with levels in healthy controls. The highest levels of free TFPI were recorded group 5. We concluded that the elevated levels of both the total and free TFPI in various phases of nephrotic syndrome add another natural anticoagulant mechanism, which will attenuate the hypercoagulability of childhood nephrotic syndrome.
KeywordsNephrotic syndrome Tissue factor pathway inhibitor TFPI Haemostasis
We are grateful to Mr. M.A. Hamid, Mr. Lugman A.G. El-Sid and Mrs. M.C. Aradillous for their technical assistance, to Mr. Amir Abdul Aziz for statistical help, and to Mrs. F. Chatila for her secretarial work. This work was supported by a grant (no. AT 18–73) from King Abdul Aziz City for Science and Technology (KACST), Riyadh, Saudi Arabia.
- 1.Livio M (1989) Hypercoagulability in the nephrotic syndrome. In: Remuzzi G, Rossi EC (eds) Haemostasis and the kidney. Butterworth, London, pp 145–152Google Scholar
- 10.Al Mugeiren MM, Gader AMA, Al-Rasheed SA, Bahakim HM, Al-Momen AK, Al-Salloum A (1995) Platelet aggregometry—dose related responses to arachidonic acid in childhood nephrotic syndrome. Platelets 6:71–74Google Scholar
- 16.Yermiahu T, Shalev H, Landau D, Dvilansky A (1996) Protein C and protein S in pediatric nephrotic patients. Sangre (Barc) 41:155–157Google Scholar
- 22.Sandset PM, Warn-Cramer BJ, Rao LVM, Maki SL, Rapaport SI (1991) Depletion of extrinsic pathway inhibitor (EPI) sensitizes rabbits to disseminated intravascular coagulation induced with tissue factor: evidence supporting a physiologic role for EPI as a natural anticoagulant. Proc Natl Acad Sci USA 88:708–712PubMedGoogle Scholar
- 25.Bregengard C, Nordfang O, Wildgoose P, Svendsen O, Hedner U, Diness V (1993) The effect of two-domain tissue factor pathway inhibitor on endotoxin-induced disseminated intravascular coagulation in rabbits. Blood Coagul Fibrinolysis 4:699–676Google Scholar
- 26.Holst J, Lindblad B, Bergqvist D, Nordfang O, Ostergaard PB, Peterson JG, Nielsen G, Hedner U (1994) Antithrombotic effect of recombinant truncated tissue factor pathway inhibitor (TFPI-161) in experimental venous thrombosis—a comparison with low molecular weight heparin. Thromb Haemost 71:214–219PubMedGoogle Scholar
- 28.Areins RAS, Moia M, Rivolta E, Ponticelli C, Mannucci PM (1999) High levels of tissue factor pathway inhibitor in patients with nephrotic proteinuria. Thromb Haemost 82:1020–1023Google Scholar
- 43.Tkaczyk M, Owczarek D, Puczko-Nogal B, Makosiej R, Rogowska-Kalisz A, Ptasnik W, Finke D (2000) Activation of coagulation cascade in children during an idiopathic nephrotic syndrome relapse. Pol Merkuriusz Lek 8:226–227Google Scholar
- 46.Loirat C, Hurtaud-Roux MF, Schlegel N, Brun P (1992) Thromboembolic complications in nephrotic syndrome. Pediatr Nephrol 6:C67Google Scholar
- 47.Svenson PJ, Dahlback B (1994) Resistance to activated protein C as a basis for venous thrombosis. N Engl J Med 330:517–522Google Scholar
- 48.Camisi S, Cavatoris F (1998) A case of deep vein thrombosis in idiopathic nephrotic syndrome with resistance to activated protein C. J Nephrol 11:76–77Google Scholar