Abstract

Atypical hemolytic uremic syndrome is the result of a spectrum of diseases. Disorders of complement regulation are the most important reasons in the etiology. It is associated with defective regulation of the alternative complement pathway in over 50% of the cases. Clinical abnormalities are related with the presence of thrombotic microangiopathy. Patients with atypical hemolytic uremic syndrome have a poor prognosis with a high mortality and morbidity in the acute phase of the disease and progression to end-stage renal disease in 50% of the cases. Various extra renal complications due to systemic thrombotic microangiopathy may occur in HUS, including neurological, pancreatic and cardiac involvement. Eculizumab is a humanized monoclonal anti-C5 antibody. It blocks the alternative complement pathway at the level of proinflammatory C5a and lytic C5b-9 complex generation. With the increase in experience, eculizumab therapy may be the first-line treatment. We do not know the optimal duration of eculizumab therapy. We also do not know in which patient a severe relapse could develop. At this moment we can suggest that eculizumab is life-saving and enhances the quality of life

Keywords: Atypical hemolytic uremic syndrome, Eculizumab, Thrombotic microangiopathy

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Copyright and license

Copyright © 2015 The Author(s). This is an open access article distributed under the Creative Commons Attribution License (CC BY), which permits unrestricted use, distribution, and reproduction in any medium or format, provided the original work is properly cited.

How to cite

1.
Gülleroğlu K, Gülleroğlu K, Baskın E. Atypical Hemolytic Uremic Syndrome. Turk J Pediatr Dis [Internet]. 2015 Dec. 1 [cited 2025 Aug. 23];9(4):286-91. Available from: https://turkjpediatrdis.org/article/view/313