Skip to main content
Log in

Difficulty in managing nephrotic syndrome-associated cerebral venous thrombosis

  • Case Report
  • Published:
CEN Case Reports Aims and scope Submit manuscript

Abstract

Thromboembolism is one of the most serious complications of nephrotic syndrome (NS). Although the occurrence of renal vein thrombosis or deep vein thrombosis is well recognized in NS patients, they rarely develop cerebral venous thrombosis (CVT). The mortality rate of CVT patients is still approximately 10%, and 6–10% of patients who survive have a severe and permanent disability. Herein, we report the case of a 26-year-old woman with multiple thrombotic risk factors, including the presence of NS, use of oral contraceptives, smoking, and alcohol consumption who developed wide-range CVT. Undetermined fraction heparin, albumin and AT-III transfusion, and direct mechanical catheter thrombectomy were insufficient for the improvement of CVT. However, CVT eventually improved along with the remission of NS by prednisolone administration. This process indicates that in the management of CVT associated with NS, it is crucial to control the activity of NS. Currently, knowledge on the treatment for NS associated with CVT is limited, and this is a subject of urgent investigation.

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.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med. 2005;352(17):1791–8.

    Article  CAS  Google Scholar 

  2. Ferro JM, Canhão P, Stam J, et al. Barinagarrementeria F. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke. 2004;35(3):664–70.

    Article  Google Scholar 

  3. Silvis SM, de Sousa DA, Ferro JM, et al. Cerebral venous thrombosis. Nat Rev Neurol. 2017;13(9):555–65.

    Article  Google Scholar 

  4. Green M, Styles T, Russell T, et al. Non-genetic and genetic risk factors for adult cerebral venous thrombosis. Thromb Res. 2018;169:15–22.

    Article  CAS  Google Scholar 

  5. Dentali F, Gianni M, Crowther MA, et al. Natural history of cerebral vein thrombosis: a systematic review. Blood. 2006;108(4):1129–34.

    Article  CAS  Google Scholar 

  6. Coutinho JM, Zuurbier SM, Stam J. Declining mortality in cerebral venous thrombosis: a systematic review. Stroke. 2014;45(5):1338–411.

    Article  Google Scholar 

  7. Kerlin BA, Ayoob R, Smoyer WE. Epidemiology and pathophysiology of nephrotic syndrome-associated thromboembolic disease. Clin J Am Soc Nephrol. 2012;7(3):513–20.

    Article  CAS  Google Scholar 

  8. Al Azzawi HF, Obi OC, Safi J, et al. Nephrotic syndrome-induced thromboembolism in adults. Int J Crit Illn Inj Sci. 2016;6(2):85–8.

    Article  Google Scholar 

  9. Bakhtawar K, Mahmoodi B. High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome: results from a large retrospective cohort study. Circulation. 2008;117(2):224–30.

    Article  Google Scholar 

  10. Cameron JS, Blandford G. The simple assessment of selectivity in heavy proteinuria. Lancet. 1966;30(2):242–7.

    Article  Google Scholar 

  11. Iwaki H, Neshige S, Hara N, et al. Cerebral venous thrombosis as a complication of nephrotic syndrome–a case report and literature review. Rinsho Shinkeigaku. 2014;54(6):495–501 (in Japanese).

    Article  Google Scholar 

  12. Zhang LJ, Zhang Z, Li SJ, et al. Pulmonary embolism and renal vein thrombosis in patients with nephrotic syndrome: prospective evaluation of prevalence and risk factors with CT. Radiology. 2014;273(3):897–906.

    Article  Google Scholar 

  13. Johannesdottir SA, Horváth-Puhó E, Dekkers OM, et al. Use of glucocorticoids and risk of venous thromboembolism: a nationwide population-based case-control study. JAMA Intern Med. 2013;173(9):743–52.

    Article  CAS  Google Scholar 

  14. Zaffanello M, Brugnara M, Fanos V, et al. Prophylaxis with AT III for thromboembolism in nephrotic syndrome: why should it be done? Int Urol and Nephrol. 2009;41(3):713–6.

    Article  CAS  Google Scholar 

  15. Nishi S, Ubara Y, Utsunomiya Y, et al. Evidence-based clinical practice guidelines for nephrotic syndrome. Clin Exp Nephrol. 2014;20(3):342–70.

    Article  Google Scholar 

  16. Radhakrishnan J, Cattran DC. The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines—application to the individual patient. Kidney Int. 2012;82(8):840–56.

    Article  Google Scholar 

  17. Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77(6):808–18.

    PubMed  Google Scholar 

  18. Saposnik G, Barinagarrementeria F, Brown RD, et al. American Heart Association Stroke Council and the Council on Epidemiology and Prevention Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2011;42(4):1158–92.

    Article  Google Scholar 

  19. Einhäupl K, Stam J, Bousser MG, et al. European Federation of Neurological Societies. EFNS guideline on the treatment of cerebral venous and sinus thrombosis in adult patients. Eur J Neurol. 2010;17(10):1229–355.

    Article  Google Scholar 

  20. Shinohara Y, Yamaguchi T. Outline of the Japanese guidelines for the management of stroke 2004 and subsequent revision. Int J Stroke. 2007;2:133–5.

    Article  Google Scholar 

  21. van Dongen CJ, van den Belt AG, Prins M, et al. Fixed dose subcutaneous low molecular weight heparins versus adjusted dose unfractionated heparin for venous thromboembolism. Cochrane Database Syst Rev. 2004;4:CD001100.

    Google Scholar 

  22. Vaziri ND, Paule P, Toohey J, et al. Acquired deficiency and urinary excretion of antithrombin III in nephrotic syndrome. Arch Intern Med. 1984;144(9):1802–3.

    Article  CAS  Google Scholar 

  23. Li SJ, Tu YM, Zhou CS, et al. Risk factors of venous thromboembolism in focal segmental glomerulosclerosis with nephrotic syndrome. Clin Exp Nephrol. 2016;20(2):212–7.

    Article  CAS  Google Scholar 

  24. Robert A, Olmer M, Sampol J, et al. Clinical correlation between hypercoagulability and thrombo-embolic phenomena. Kidney Int. 1978;31(3):830–5.

    Article  Google Scholar 

  25. Cherng SC, Huang WS, Wang YF, et al. The role of lung scintigraphy in the diagnosis of nephrotic syndrome with pulmonary embolism. Clin Nucl Med. 2000;25:167–72.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Shu Wakino.

Ethics declarations

Conflict of interest

The authors have declared that no conflict of interests exists.

Ethical approval

This article does not contain any studies with human participants performed by any of the authors.

Informed consent

The authors declare that they have obtained consent from the patient’s family discussed in the report.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Nakayama, T., Mitsuno, R., Torimitsu, T. et al. Difficulty in managing nephrotic syndrome-associated cerebral venous thrombosis. CEN Case Rep 10, 132–138 (2021). https://doi.org/10.1007/s13730-020-00520-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13730-020-00520-7

Keywords

Navigation