Abstract
Background
Thromboembolism is a rare life-threatening complication of childhood nephrotic syndrome.
Methods
We present the clinical profile and outcome of 34 children with 35 events of thromboembolic complications with nephrotic syndrome.
Results
Cerebral venous thrombosis (CVT) was the commonest complication seen in 11 (31.4 %) children followed by pulmonary thromboembolism and deep venous thrombosis in 9 (25.7 %) and 6 (16.6 %) children, respectively. Arterial thrombosis resulting in central nervous system infarcts was observed in 7 (20 %) children and 2 children had thrombosis of the peripheral arteries. Episodes were equal in steroid-resistant nephrotic syndrome and steroid-dependent nephrotic syndrome groups. Most of the thromboembolic complications occurred with relapse but 11.4 % of children developed intracranial thrombosis during remission. The most sensitive symptom of CVT was persistent headache while unexplained respiratory distress and hypoxemia pointed towards pulmonary thromboembolism. Hypoalbuminemia was seen in 82.8 % of children, while concurrent infection was seen in 31.4 %. Coexistence of genetic prothrombotic condition was identified and merits evaluation. Early heparin therapy followed by oral anticoagulants resulted in complete recovery in 91.1 % of children. Death occurred in 3 (8.5 %) children and autopsy revealed pulmonary thromboembolism in 2 children.
Conclusion
Venous and arterial thrombotic complications can occur in children with nephrotic syndrome. A high index of suspicion is required as the clinical features may be subtle. Neuroimaging and angiographic techniques help in confirming diagnosis. Early aggressive heparin therapy followed by oral anticoagulants is necessary for a favorable outcome.
Similar content being viewed by others
References
Niaudet P. Steroid resistant nephrotic syndrome. In: Niaudet P, Aver ED, Harmon WE, editors. Pediatric nephrology. Philadelphia: Lippincott Williams and Wilkins; 2004. p. 557–74.
Indian Pediatric Nephrology Group, Indian Academy of Pediatrics. Consensus statement on management of steroid sensitive nephrotic syndrome. Indian Pediatr. 2001;38:975–86.
Indian Pediatric Nephrology Group, Indian Academy of Pediatrics, Bagga A, Ali U, Banerjee S, Kanitkar M, Phadke KD, Senguttuvan P, et al. Management of steroid sensitive nephrotic syndrome: revised guidelines. Indian Pediatr. 2008;45:203–14.
Indian Society of Pediatric Nephrology, Gulati A, Bagga A, Gulati S, Mehta KP, Vijayakumar M. Management of steroid resistant nephrotic syndrome. Indian Pediatr. 2009;46:35–47.
Suri D, Gupta N, Morigeri C, Saxena A, Manoj R. Chylopericardial tamponade secondary to superior vena cava thrombosis in a child with nephrotic syndrome. Pediatr Nephrol. 2009;24:1234–5.
Hoyer PF, Gonda S, Barthels M, Krohn HP, Brodehl J. Thromboembolic complications in children with nephrotic syndrome. Risk and incidence. Acta Paediatr Scand. 1986;75:804–10.
Rabelink JT, Zwaginga JJ, Koomans HA, Sixma JJ. Thrombosis and hemostasis in renal disease. Kidney Int. 1994;46:287–96.
Tinaztepe K, Buyan N, Tinaztepe B, Akkok N. The association of nephrotic syndrome and renal vein thrombosis: a clinicopathological analysis of eight pediatric patients. Turk J Pediatr. 1989;31:1–18.
Lilova MI, Velkovski IG, Topalov IB. Thromboembolic complications in children with nephrotic syndrome in Bulgaria (1974–1996). Pediatr Nephrol. 2000;15:74–8.
Andrew M, Brooker LA. Hemostatic complications in renal disorder of the young. Pediatr Nephrol. 1996;10:88–99.
Egli F, Eimiger P, Stalder G. Thromboembolism in the nephrotic syndrome (abstract). Pediatr Res. 1973;8:803.
Mehls O, Andrassay K, Koderisch J, Herzog U, Ritz E. Hemostasis and thromboembolism in children with nephrotic syndrome: differences from adult. J Pediatr. 1987;110:862–7.
Schlegel N. Thromboembolic risks and complications in nephrotic children. Semin Thromb Hemost. 1997;23:271–80.
Fabri D, Belangero VM, Annichino-Bizzacchi JM, Arruda VR. Inherited risk factors for thrombophilia in children with nephrotic syndrome. Eur J Pediatr. 1998;157:939–42.
Martinez Ara J, Gomez Rioja R, Rinon C, Garcia Munoz MS, Ruiz Caravaca ML, Miguel JL. Prevalence of genetic prothrombotic factors (factor V Leiden and II20210 prothrombin mutation) in glomerular nephropathies with or without thrombosis. Nefrologia. 2000;20:139–44.
Schöning M, Klein R, Krägeloh-Mann I, Falck M, Bien S, Berg PA, et al. Antiphospholipid antibodies in cerebrovascular ischemia and stroke in childhood. Neuropediatrics. 1994;25(1):8–14.
Fluss J, Geary D, de Veber G. Cerebral sinovenous thrombosis and idiopathic nephrotic syndrome in childhood: report of four new cases and review of the literature. Eur J Pediatr. 2006;165:709–16.
Ishikura K, Ikeda M, Hamasaki Y, Hataya H, Nishimura G, Hiramoto R, Honda M. Nephrotic state as a risk factor for developing posterior reversible encephalopathy syndrome in paediatric patients with nephrotic syndrome. Nephrol Dial Transplant. 2008;23:2531–6.
Ishikura K, Ikeda M, Hamasaki Y, Hataya H, Shishido S, Asanuma H, Nishimura G, Hiramoto R, Honda M. Posterior reversible encephalopathy syndrome in children: its high prevalence and more extensive imaging findings. Am J Kidney Dis. 2006;48:231–8.
Huang J, Yang J, Ding J. Pulmonary embolism associated with nephrotic syndrome in children: a preliminary report of 8 cases. Chin Med J (Engl). 2000;113:251.
Deshpande PV, Griffiths M. Pulmonary thrombosis in steroid-sensitive nephrotic syndrome. Pediatr Nephrol. 2005;20:665–9.
Mittal BR, Singh S, Bhattacharya A, Prasad V, Singh B. Lung scintigraphy in the diagnosis and follow-up of pulmonary thromboembolism in children with nephrotic syndrome. J Clin Imaging. 2005;29:313–6.
Stein PD, Gottschalk A, Sostman HD, Chenevert TL, Fowler SE, Goodman LR, et al. Methods of prospective investigation of pulmonary embolism diagnosis III (PIOPED III). Semin Nucl Med. 2008;38:462–70.
Witz M, Lehman J, Shnaker A, Korzets Z. Acute subclavian and brachial artery thrombosis as a complication of the nephrotic syndrome. Isr Med Assoc J. 2004;6:441–2.
Siddiqi FA, Tepler J, Fantini GA. Acquired protein S and antithrombin III deficiency caused by nephrotic syndrome: an unusual cause of graft thrombosis. J Vasc Surg. 1997;25:576–80.
Hanna J, Truemper E, Burton E. Superior vena cava thrombosis and chylothorax: relationship in pediatric nephritic syndrome. Pediatr Nephrol. 1997;11:20–2.
Chugh KS, Malik N, Uberoi HS, Gupta VK, Aggarwal ML, Singhal PC, et al. Renal vein thrombosis in nephrotic syndrome—a prospective study and review. Postgrad Med J. 1981;57:566–70.
Trew PA, Biava CG, Jacobs RP, Hopper J Jr. Renal vein thrombosis in membranous glomerulonephropathy: incidence and association. Medicine. 1978;57:69–82.
Velasquez Forero F, Garcia Prugue N, Ruiz Morales N. Idiopathic nephrotic syndrome of the adult with asymptomatic thrombosis of the renal vein. Am J Nephrol. 1988;8:457–62.
Wagoner RD, Stanson AW, Holley KE, Winter CS. Renal vein thrombosis in idiopathic membranous glomerulopathy and nephrotic syndrome: incidence and significance. Kidney Int. 1983;23:368–74.
Proceedings of the Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy: evidence-based guidelines. Chest 2004;126:172S–696S.
Conflict of interest
All the authors have declared no competing interest.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Suri, D., Ahluwalia, J., Saxena, A.K. et al. Thromboembolic complications in childhood nephrotic syndrome: a clinical profile. Clin Exp Nephrol 18, 803–813 (2014). https://doi.org/10.1007/s10157-013-0917-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10157-013-0917-2