Diagnostic clues in atypical hemolytic uremic syndrome: a case report

Authors

  • M. Luquin Irigoyen Servicio de Análisis Clínicos. Hospital Universitario de Navarra. Servicio Navarro de Salud-Osasunbidea. Pamplona. España.
  • C. Armendariz Brugos Servicio de Análisis Clínicos. Hospital Universitario de Navarra. Servicio Navarro de Salud-Osasunbidea. Pamplona. España.
  • M. Vallejo Ruiz Servicio de Análisis Clínicos. Hospital Universitario de Navarra. Servicio Navarro de Salud-Osasunbidea. Pamplona. España.

DOI:

https://doi.org/10.23938/ASSN.1006

Keywords:

Atypical hemolytic-uremic síndrome, Microangiopathic hemolytic anemia, Complement pathway alternative, Complement System Proteins, Eculizumab

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a clinical entity characterized by non-immune hemolytic anemia, thrombocytopenia and renal failure, in which lesions are mediated by a systemic thrombotic microangiopathy. It is a rare pathology whose origin is a complement system deregulation due to mutations in its genes that lead to uncontrolled activation of C5 and the formation of the membrane attack complex.­ Its correct diagnosis allows us to prescribe the treatment based on Eculizumab, a C5 inhibitor.

We report the case of a pregnant patient with aHUS, with the aim of highlighting the importance of early differential diagnosis to establish an early and effective treatment of this pathology. The pathophysiology, diagnosis and genetic study are updated, as well as the therapeutic management of aHUS.

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References

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Published

2022-08-17

How to Cite

Luquin Irigoyen, M., Armendariz Brugos, C., & Vallejo Ruiz, M. (2022). Diagnostic clues in atypical hemolytic uremic syndrome: a case report. Anales Del Sistema Sanitario De Navarra, 45(2), e1006. https://doi.org/10.23938/ASSN.1006

Issue

Vol. 45 No. 2 (2022): May - August 2022

Section

Clinical notes

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