Hemolytic Uremic Syndrome: Single Centre Experience

Year 2018, , 12 - 18, 01.01.2018

Can Huzmeli , Hatice Terzi Ferhan Candan Meryem Timucin Ayse Seker Mehmet Sencan Mansur Kayatas

https://doi.org/10.5455/umj.20180304114653

Abstract

Introduction: Hemolytic uremic syndrome HUS is a clinical syndrome characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. In this study, we aimed to evaluate patients diagnosed with HUS in our clinic. Method: Patients who were diagnosed with HUS as clinic and laboratory between August 2012 and October 2017 were included in our nephrology clinic. Biochemistry, haemogram, ANA, anti-Ds DNA, p-ANCA, c-ANCA, anti-GBM antibody, C3, C4 of patients were studied. In some cases, ADAMTS13 was studied and renal biopsy was performed. The demographic features, clinics, and treatments of our cases were reviewed. Results: A total of 18 patients were enrolled in the study, the average age of the patients was 48.3 21-82 and 10 of the cases were female and 8 of the cases were male. Among the etiologic causes of HUS in patients; the complement factor B mutation, complement factor H polymorphism, breast cancer, herbal medicine, infections and idiopathic were detected. Primer glomerulonephritis was detected in 6 cases of renal biopsy. Conclusion: Clinical and laboratory remission was obtained in 13 patients. End-stage renal failure developed in 3 of our patients.

Keywords

Acute kidney damage, Hemolytic anemia, thrombocytopenia, eculizumab

References

• 1. Amirlak I, Amirlak B. Haemolytic uraemic syndrome: An overview. Nephrology 2006;11:213–218.
• 2. Canpolat N. Hemolytic uremic syndrome. Turk Pediatri Ars. 2015; 50: 73-82.
• 3. Noris M, Remuzzi G. Hemolytic uremic syndrome. J Am Soc Nephrol. 2005; 16: 1035–1050.
• 4. Blaser MJ. Deconstructing a lethal food borne epidemic. N Engl J Med. 2011; 365: 1835-1836.
• 5. Salvadori M, Bertoni E. Update on haemolytic uremic syndrome: Diagnostic and therapeutic recommendations. World J Nephrol 2013; 2(3): 56-76.
• 6. Sharma AP, Greenberg CR, Prasad AN, Prasad C. Hemolytic uremic syndrome (HUS) secondary to cobalamin C (cblC) disorder. Pediatr Nephrol 2007;22:2097-2103.
• 7. Waters AM, Kerecuk L, Luk D, Haq MR, Fitzpatrick MM, Gilbert RD, et al. Hemolytic uremic syndrome associated with invasive pneumococcal disease: the United kingdom experience. J Pediatr. 2007;151:140-144.
• 8. Allen U, Licht C. Pandemic H1N1 influenza A infectionand (atypical) HUS more than just another trigger? Pediatr Nephrol. 2011;26:3-5.
• 9. Besbas N, Karpman D, Landau D, Loirat C, Proesmans W, Remuzzi G, et al. A classification of haemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders. Kidney Int. 2006;70(3):423-431.
• 10. Frank C, Werber D, Cramer JP, Askar M, Faber M, an der Heiden M, et al. Epidemic profile of Shiga-toxin- producing Escherichia coli O104:H4 outbreak in Germany. N Engl J Med. 2011;365: 1771-1780.
• 11. Srivastava RN, Moudgil A, Bagga A, Vasudev AS. Hemolytic uremic syndrome in children in northern India. Pediatr Nephrol. 1991;3:284-288.
• 12. Noris M, Remuzzi G. “Genetic abnormalities of complement regulators in haemolytic uremic syndrome: how do they affect patient management?” Nature Clinical Practice Nephrology. 2005;1:2–3.
• 13. Kavanagh D and Goodship T. “Genetics and complement in atypical HUS,” Pediatric Nephrology. 2010;25:2431–2442.
• 14. Botto M, Kirschfink M, Macor P, Pickering MC, Würzner R, Tedesco F. Complement in human disease: Lessons from complement deficiencies. Mol Immunol. 2009;46:2774-2783.
• 15. Pettigrew HD, Teuber SS, Gershwin ME. Clinical significance of complement deficiencies. Ann N Y Acad Sci. 2009;1173: 108-123.
• 16. Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S, et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010;5:1844-1859.
• 17. Chang A, Kowalewska J, Smith KD, Nicosia RF, Alpers CE. A clinicopathologic study of thrombotic microangiopathy in the setting of IgA nephropathy. Clin Nephrol. 2006;66:397- 404.
• 18. Zheng XL, Sadler JE. Pathogenesis of thrombotic micro angiopathies. Annu Rev Pathol 2008;3:249-277.
• 19. Morita S, Sakai T, Okamoto N, Funabiki A, Okada Y, Hasegawa Y, et al. Hemolytic uremic syndrome associated with immunglobulin A nephropathy: a case report and review of cases of haemolytic uremic syndrome with glomerular disease. Int Med 1999;38:495-499.
• 20. Servais A, Frémeaux-Bacchi V, Lequintrec M, Salomon R, Blouin J, Knebelmann B, et al. Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome. J Med Genet 2007;44:193-199.
• 21. Safdar N, Said A, Gangnon RE, Maki DG. Risk of haemolytic uremic syndrome after anti- biotic treatment of Escherichia coli O157: H7 enteritis. JAMA. 2002;288:996-1001.
• 22.Advice of the German Society of Nephrology on the use of Ecilizumab during the 2011 EHEC HUS outbreak 2011. http://www.dgfn.eu/aktuell/ehec-informationen/fuer-dasfachpublikum/advice-on-the-useof-ecilizumab.html.