Skip to main content
Log in

Recurrent renal vein thrombosis and renal failure associated with antithrombin-III deficiency

  • Original Article
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

This paper describes a healthy 13-year-old girl presenting with acute flank pain and anuria due to renal vein thrombosis. A similar spontaneous and unexplained thrombosis resulted in the loss of the contralateral kidney 1 year earlier. Urgent surgical thrombectomy and anticoagulation resulted in moderate recovery of renal function. Predisposition to venous thrombosis in this child was secondary to a marked familial deficiency of circulating antithrombin-III. An early diagnosis of this condition is essential for the formulation of preventive measures and may lead to specific therapeutic intervention at the onset of acute thrombotic complications.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Rosenberg RD, Rosenberg JS (1984) Natural anticoagulant mechanisms. J Clin Invest 74: 1–6

    Google Scholar 

  2. Lusher JM (1987) Diseases of coagulation: the fluid phase. In: Nathan DG, Oski FA (eds) Hematology of infancy and childhood. Saunders, Philadelphia, p 1325

    Google Scholar 

  3. Comp PC (1986) Hereditary disorders predisposing to thrombosis. Prog Hemost Thromb 8: 71–102

    Google Scholar 

  4. Egeberg O (1965) Inherited antithrombin deficiency causing thrombophilia. Thromb Diath Haemorrh 13: 516–530

    Google Scholar 

  5. Van Der Meer J, Stoepman-Van Dalen EA, Jansen JMS (1973) Antithrombin-III deficiency in a Dutch family. J Clin Pathol 26: 532–538

    Google Scholar 

  6. Thaler E, Lechner K (1981) Antithrombin-III deficiency and thromboembolism. Clin Haematol 10: 369–390

    Google Scholar 

  7. Gruenberg JC, Smallridge RC, Rosenberg RD (1975) Inherited antithrombin-III deficiency causing mesenteric venous infarction. Ann Surg 181: 791–794

    Google Scholar 

  8. Hoyer PF, Gonda S, Barthels M, Krohn HP, Brodehl J (1986) Thromboembolic complications in children with nephrotic syndrome. Acta Paediatr Scand 75: 804–810

    Google Scholar 

  9. Mehls I, Andrassy K, Koderisch J, Herzog U, Ritz E (1987) Hemostasis and thromboembolism in children with nephrotic syndrome: differences from adults. J Pediatr 110: 862–867

    Google Scholar 

  10. Marciniak E, Farley CH, DeSimone PA (1974) Familial thrombosis due to antithrombin III deficiency. Blood 43: 219–231

    Google Scholar 

  11. Von Kaulla E, Von Kaulla KN (1967) Antithrombin III and diseases. Am J Clin Pathol 48: 69–80

    Google Scholar 

  12. Lieberman E, Heuser E, Gilchrist GS, Donnell GN, Landing BN (1968) Thrombosis, nephrosis, and corticosteroid therapy. J Pediatr 73: 320–328

    Google Scholar 

  13. Trygstad CW, McCabe E, Francyk WP, Crummy AB (1970) Renal vein thrombosis and the nephrotic syndrome: a case report with protein selectivity studies. J Pediatr 76: 861–866

    Google Scholar 

  14. Llach F, Arieff AI, Massry SG (1975) Renal vein thrombosis and nephrotic syndrome. Ann Intern Med 83: 8–14

    Google Scholar 

  15. Kauffmann RH, Veltkamp JJ, Van Tilburg NH, Van Es LA (1978) Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome. Am J Med 65: 607–613

    Google Scholar 

  16. Llach F (1985) Hypercoagulability, renal vein thrombosis, and other thrombotic complications of nephrotic syndrome. Kidney Int 28: 429–439

    Google Scholar 

  17. Vaziri ND, Paule P, Toohey J, Hung E, Alikhani S, Darwish R, Pahl MV (1984) Acquired deficiency and urinary excretion of antithrombin III in nephrotic syndrome. Arch Intern Med 144: 1802–1803

    Google Scholar 

  18. Bernard DB (1988) Extrarenal complications of the nephrotic syndrome. Kidney Int 33: 1184–1202

    Google Scholar 

  19. Rose WF (1982) Treatment of paroxysmal nocturnal hemoglobinuria. Blood 60: 20–23

    Google Scholar 

  20. Fein RL, Chait A, Leviton A (1968) Renal vein thrombectomy for the treatment of renal vein thrombosis associated with the nephrotic syndrome. J Urol 99: 1–13

    Google Scholar 

  21. Balabanian MB, Schnetzler DE, Kaloyanides GJ (1973) Nephrotic syndrome, renal vein thrombosis and renal failure. Am J Med 54: 768–776

    Google Scholar 

  22. Rowe JM, Rasmussen RL, Mader SL, Dimarco PL, Cockett ATK, Marder VJ (1984) Successful thrombolytic therapy in two patients with renal vein thrombosis. Am J Med 77: 1111–1114

    Google Scholar 

  23. DeStefano V, Leone G, DeCarolis S, Ferrelli R, Di Donfrancesco A, Moneta E, Bizzi B (1988) Management of pregnancy in women with antithrombin III congenital defect: report of four cases. Thromb Haemost 59: 193–196

    Google Scholar 

  24. Shibuya A, Ninomiya H, Nakazawa M, Nagasaw T, Yoda Y, Abe T (1988) Oxymetholone therapy in patients with familial antithrombin III deficiency. Thromb Haemost 60: 495–497

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ellis, D. Recurrent renal vein thrombosis and renal failure associated with antithrombin-III deficiency. Pediatr Nephrol 6, 131–134 (1992). https://doi.org/10.1007/BF00866291

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00866291

Key words

Navigation