A Study of Fibrinogen and Fibrinolysis in 10 Adults with Nephrotic Syndrome

S Gandrille; M H Jouvin; P Toulon; P Remy; J N Fiessinger; M Roncato; N Moatti; M Aiach

doi:10.1055/s-0038-1647513

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 1988; 59(03): 445-450
DOI: 10.1055/s-0038-1647513

Original Article

Schattauer GmbH Stuttgart

A Study of Fibrinogen and Fibrinolysis in 10 Adults with Nephrotic Syndrome

Authors

S Gandrille

^*The Laboratoire de Biochimie et Hémostase, Hôpital Broussais, Paris, France
M H Jouvin

^**The Service de Néphrologie, Hôpital Broussais, Paris, France
P Toulon

^*The Laboratoire de Biochimie et Hémostase, Hôpital Broussais, Paris, France
P Remy

^**The Service de Néphrologie, Hôpital Broussais, Paris, France
J N Fiessinger

^***The Chaire de clinique médicale et de Pathologie vasculaire, Hôpital Broussais, Paris, France
M Roncato

^*The Laboratoire de Biochimie et Hémostase, Hôpital Broussais, Paris, France
N Moatti

^*The Laboratoire de Biochimie et Hémostase, Hôpital Broussais, Paris, France
M Aiach

^*The Laboratoire de Biochimie et Hémostase, Hôpital Broussais, Paris, France

Abstract

PDF Download

Summary

In 10 patients with nephrotic syndrome (NS), the coagulation inhibitors, the fibrinolytic system and several functions of the fibrinogen-fibrin molecule were studied.

Among the coagulation inhibitors, only antithrombin III (AT III) was found decreased and correlated with serum-albumin levels. Venous occlusion test provoked a normal tissue plasminogen activator (tPA) release in all patients. The plasminogen activator inhibitor (PAI) had an increased activity in 5 out of the 10 patients.

Thrombin and reptilase times were found abnormal in most patients. The thrombin time (TT) prolongation correlated with serum albumin levels and was corrected by adding purified albumin.

The fibrinogen was purified from each of the 10 patients’ plasma. Only 2 of them showed abnormal polymerization in purified system, suggesting dysfibrinogenaemia. Other functions (thrombin binding, tPA stimulating activity, lysis by purified plasmin) were found normal except in one of the 2 patients with dysfibrinogenaemia whose fibrinogen lysis by plasmin was delayed.

It is concluded that an abnormal fibrinogen molecule is not the most frequent explanation for thrombin time prolongation in NS.

Keywords

Nephrotic syndrome - Fibrinolytic system - Fibrinogen - Albumin

PDF (2042 kb)

References

References
1 Kanfer A, Kleinknecht D, Broyer M, Josso F. Coagulation studies in 45 cases of nephrotic syndrome without uremia. Thromb Diathes Haemorrh 1970; 24: 562-571
2 Abeshouse BS. Thrombosis and thrombophlebitis of the renal veins. Urol Cutan Rev 1945; 49: 661-675
3 Llach F, Koffler A, Massry SG. Renal vein thrombosis and the nephrotic syndrome. Nephron 1977; 19: 65-68
4 Rosenberg RD. Actions and interactions of antithrombin and heparin. N Engl J Med 1975; 292: 146-151
5 Clouse LH, Comp PC. The regulation of hemostasis: Protein C system. N Engl J Med 1986; 314: 1298-1304
6 Marder VJ, Shulman NR, Carroll WR. High molecular weight derivatives of human fibrinogen produced by plasmin. I. Physicochemical and immunological characterization. J Biol Chem 1969; 244: 2111-2119
7 Liu CY, Nossel HL, Kaplan KL. The binding of thrombin by fibrin. J Biol Chem 1979; 254: 10421-10425
8 Winter CS, Wagoner RD, Bowie EJ W, Owen CA. Dysfibrinogenemia and hypercoagulability in patients with membranous glomerulopathy. Thromb Haemostas 1979; 42: 67 (Abstr)
9 Clauss A. Germungsphysiologische Schnell-Methode zur Bestimmung des Fibrinogen. Acta Haematol 1957; 17: 237-246
10 Biggs RM. In: Human Blood Coagulation, Haemostasis and Thrombosis. Blackwell Sci Publ; Oxford: 1972
11 Aiach M, Leon M, Michaud A, Capron L. Adaptation of synthetic peptide substrate-based assays on a discrete analyzer. Semin Thromb Haemostas 1983; 9: 206-216
12 Boyer C, Rothschild C, Wolf H, Amiral J, Meyer D, Larrieu MJ. A new method for the estimation of protein C by ELISA. Thromb Res 1984; 36: 579-589
13 Toulon P, Jacquot C, Capron L, Frydman MO, Vignon D, Aiach M. Antithrombin III and heparin cofactor II in patients with chronic renal failure undergoing regular hemodialysis. Thromb Haemostas 1987; 57: 263-268
14 Gandrille S, Priollet P, Capron L, Roncato M, Fiessinger JN, Aiach M. Association of inherited dysfibrinogenaemia and protein C deficiency in two unrelated families. Br J Haematol 1988; 68: 329-337
15 Kazal LA, Amsel S, Miller OP, Tocantins LM. The preparation and some properties of fibrinogen precipited from human plasma by glycin. Proc Soc Exp Biol Med 1963; 113: 989-994
16 Anglés-Cano E. A spectrophotometric solid-phase fibrin-tissue plasminogen activator activity assay (SOFIA-tPA) for high-fibrin affinity tissue plasminogen activators. Anal Biochem 1986; 153: 201-210
17 Haverkate F, Koopman J, Kluft C, D’Angelo A, Cattaneo M, Mannucci PM. Fibrinogen Milano II. A congenital dysfibrinogenemia associated with juvenile arterial and venous thrombosis. Thromb Haemostas 1986; 55: 131-135
18 Mosesson MW, Denninger MH, Menache D. Hydrolysis of fibrinogen Paris I by plasmin. Thromb Res 1976; 9: 115-121
19 Aminoff D. Methods for the quantitative estimation of N-acetyl-neuraminic acid and their application to hydrolysates of sialomucoids. Biochem J 1961; 81: 384-392
20 Kluft C. Studies on the fibrinolytic system in human plasma: Quantitative determination of plasminogen activators and pro-activators. Thromb Haemostas 1979; 41: 365-383
21 Anglés-Cano E, Boutière B, Arnoux D, Masson C, Contant G, Benchimol P, Sampol J. Release pattern of the vascular plasminogen activator and its inhibitor in human postvenous occlusion plasma as assessed by a spectrophotometric solidphase fibrin-tPA activity assay. Thromb Haemostas 1987; 58: 843-849
22 Contant G, Martinoli JL, Anglés-Cano E. Determination of plasminogen activator inhibitory activity (PAI) in human plasma. Thromb Res. (submitted)
23 Marciniak E, Gockerman JP. Heparin-induced decrease in antithrombin III. Lancet 1977; 1: 581-84
24 Kauffmann RH, Veltkamp JJ, Van Tilburg NH, Van Es LA. Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome. Am J Med 1978; 65: 607-613
25 Thaler E, Balzar E, Kopsa M, Pinggera WF. Acquired antithrombin III deficiency in patients with glomerular proteinuria. Haemostasis 1978; 7: 257-272
26 De Stefano V, Triolo L, De Martini D, Ferrelli R, Mori P, Leone G. Antithrombin III loss in patients with nephrotic syndrome or receiving continuous ambulatory peritoneal dialysis. Evidence of inactive antithrombin III in urine of patients with nephrotic syndrome J Lab Clin Med 1987; 109: 550-555
27 Vigano D'Angelo, D’Angelo A, Kaufman CE, Sholer C, Esmon CT, Comp PC. Protein S deficiency occurs in the nephrotic syndrome. Ann Intern Med 1987; 107: 42-47
28 Jorgensen M, Bonnevie-Nielsen V. Increased concentration of the fast-acting plasminogen activator inhibitor in plasma associated with familial venous thrombosis. Br J Haematol 1987; 65: 175-180
29 Juhan-Vague I, Valadier J, Alessi MC, Aillaud MF, Ansaldi J, Philip-Joet C, Holvoet P, Serradimigni A, Collen D. Deficient t-PA release and elevated PA inhibitor levels in patients with spontaneous or recurrent deep venous thrombosis. Thromb Haemostas 1987; 57: 67-72
30 Hamsten A, Wiman B, De Faire U, Blomback M. Increased plasma levels of a rapid inhibitor of tissue plasminogen activator in young survivors of myocardial infarction. N Engl J Med 1985; 313: 1557-1563
31 Sprengers ED, Kluft C. Plasminogen activator inhibitors. Blood 1987; 69: 381-387
32 Martinez J, Palascak JE, Kwasniak D. Abnormal sialic acid content of the dysfibrinogenemia associated with liver disease. J Clin Invest 1978; 61: 535-538
33 Sandbjerg MHansen, Schousboe I. An abnormal fibrinogen (Copenhagen II) with increased sialic acid content and normal liver function. Scand J Haematol 1984; 33: 9-14
34 Mosesson MW, Wautier JL, Amrani D, Dervichiqn M, Catlan D. Evidence for circulating fibrin in familian Mediterranean fever. J Lab Clin Med 1982; 99: 559-567