Coagulation Factors in Nephrotic Syndrome

Abstract

Nephrotic syndrome (NS) is associated with several disorders of hemostasis: thrombocytosis and platelet hyperaggregability; increased plasma levels of factors V and VIII, and of fibrinogen with blood hyperviscosity; decreased plasma levels of natural anticoagulants: free protein S, and antithrombin III compensated by increased levels of α₂-macroglobulin; lowered fibrinolytic activity. Intensity of hypercoagulability is related to the degree of hypoalbuminemia; however, the role of hypercoagulability in the increased incidence of thromboembolic events, including renal vein thrombosis, is not proved. Clotting disorders are due to urinary losses of anticoagulants or to increased liver synthesis of procoagulants stimulated by hypoalbuminemia. Moreover, changes in clotting factors levels may be due to intravascular thrombin formation (marked by increased plasma levels of fibrinopeptide A). During active phases of glomerulonephritides (GN) with NS, thrombin formation might in fact arise in glomeruli, following activation of the glomerular hemostasis system. Isolated glomeruli from human crescentic GN, rabbit nephrotoxic GN and rat HgCl₂ autoimmune GN produce excessive amounts of procoagulant (tissue factor) activity (PCA). Sequential studies of the self-limited HgCl₂ GN showed that glomerular PCA, proteinuria and glomerular fibrin deposits peaked concomitantly at the acme of the disease, suggesting that immunologically mediated glomerular damage had triggered the extrinsic coagulation pathway.

Multiple venous thrombosis and massive pulmonary artery thrombus as the presenting features of steroid-responsive nephrotic syndrome

“…In addition, the insertion of an inferior vena caval filter did not halt the thrombotic alterations in the coagulation inhibitors (deficiency of ATIII, often, but not always, reduced functional levels process and caval thrombosis developed. The net effect of heparin in the presence of a low concentration of of protein C and S despite increased total levels); and platelet dysfunction [11,12]. It has been shown that all antithrombin III in the nephrotic state may therefore be insufficient or even adverse in terms of thrombin these disturbances have a general tendency to correlate with the degree of hypoalbuminaemia.…”

Intraventricular and Brachial Artery Thrombosis in Nephrotic Syndrome

“…Increased levels of high-molecularweight coagulation factors such as fibrinogen, V, VII, VIII and X have been documented [18][19][20] and this increase is thought to result from increased hepatic synthesis of these factors, stimulated by hypoalbuminemia [21]. Factors IX, XI and XII are decreased possibly due to their urinary losses being of small molecular size [22][23].…”

Enhanced Expression of Plasminogen Activator Inhibitor 1 in Patients with Nephrotic Syndrome

“…Since Kanfer [1]reported various disorders of the hemostatic system in patients with nephrotic syndrome, studies have attempted to elucidate the mechanisms of this hypercoagulable state. Alterations in blood coagulation factors, anticoagulants, and platelets have been reported.…”