Acta Scientific Medical Sciences (ISSN: 2582-0931)

Case Report Volume 4 Issue 5

Atypical Hemolytic Uremic Syndrome in a One-Year Old Male Patient, Successfully Treated with Eculizumab

Henri Fero1, Juna Musa2*, Diamant Shtiza3, Ergys Cuka4, Loran Rakovica5, Blina Abdullahu1, Klejda Hoxha1, Fareeha Nasir6 and Ali Guy7

1Medical Doctor, University of Hospital Center Mother Teresa, Tirane, Albania
2Postdoctoral Research Fellow, Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA
3Professor Associate, Department Pediatrician Nephrology, University of Hospital Mother Teresa, Tirane, Albania
4Medical Doctor, University of Hospital Center Mother Teresa, Tirane, Albania
5Medical Doctor, University of Hospital in Prishtina, Kosovo
6Intern, Department of Surgery, Christian Hospital Quetta, Pakistan
7Department of Physical Medicine and Rehabilitation, School of Medicine - NUY Medical Center, New York, USA

*Corresponding Author: Juna Musa, Postdoctoral Research Fellow, Department of Surgery, Mayo Clinic, Minnesota, USA.

Received: April 12, 2020; Published: April 27, 2020

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Abstract

  Atypical hemolytic uremic syndrome is a rare form of thrombotic microangiopathies resulting from various genetic mutations of the complement factors. Thrombotic microangiopathies include Thrombotic thrombocytopenic purpura, primary HUS further divided into typical HUS or Shiga-toxin related HUS, atypical HUS and secondary forms of HUS. The most common clinical features of the Hemolytic Uremic Syndrome are: kidney failure, thrombocytopenia resulting in hemorrhagic phenomena and also intravascular hemolytic anemia. Most commonly HUS is a diagnosis made from excluding all other plausible causes. HUS predominantly affects pediatric ages.

Keywords: Atypical Hemolytic Uremic Syndrome; Eculizumab; Thrombotic Microangiopathy

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References

  1. Noris M and Remuzzi G. “Hemolytic uremic syndrome”. Journal of the American Society of Nephrology 4 (2005): 1035.
  2. Nester CM., et al. “Atypical aHUS: state of the art”. Molecular Immunology 1 (2015): 31-42.
  3. Noris M and Remuzzi G. “Atypical hemolytic-uremic syndrome”. New England Journal of Medicine 17 (2009): 1676-1687.
  4. Goodship TH., et al. “Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a Kidney”. Kidney International3 (2017): 539-551.
  5. AR Constantinescu., et al. “Non-enteropathic hemolytic uremic syndrome: causes and short-term course”. American Journal of Kidney Diseases 6 (2004): 976-982.
  6. AL Sellier-Leclerc., et al. “Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome”. Journal of the American Society of Nephrology 8 (2007): 2392-2400.
  7. M Sullivan., et al. “Epidemiological approach to identifying genetic predispositions for atypical hemolytic uremic syndrome”. Annals of Human Genetics 1 (2010): 17-26.
  8. Legendre CM., et al. “Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome”. New England Journal of Medicine 23 (2013): 2169-2181.
  9. Greenbaum LA., et al. “Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome”. Kidney International 3 (2016): 701-711.
  10. Hayes W., et al. “Eculizumab hepatotoxicity in pediatric aHUS”. Pediatric Nephrology 5 (2015): 775-781.
  11. Noris M., et al. “Dynamics of complement activation in aHUS and how to monitor eculizumab therapy”. Blood 11 (2014): 1715-1726.
  12. Caprioli J., et al. “Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome”. Blood4 (2006): 1267-1279.
  13. Noris M., et al. “Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype”. Clinical Journal of the American Society of Nephrology 10 (2010): 1844-1859.
  14. Collins Allan J., et al. "Excerpts from the US Renal Data System 2009 Annual Data Report". American Journal of Kidney Diseases 1 (2010): S1-S7.
  15. European Renal Association- European Dialysis and Transplant Association Registry. “ERA-EDTA Registry Annual Report 2009”. Amsterdam, The Netherlands: Academic Medical Center Department of Medical Informatics (2011).
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Citation

Citation: Juna Musa., et al. “Atypical Hemolytic Uremic Syndrome in a One-Year Old Male Patient, Successfully Treated with Eculizumab". Acta Scientific Medical Sciences 4.5 (2020): 84-86.




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