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Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations. | LitMetric

Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations.

World J Nephrol

Maurizio Salvadori, Elisabetta Bertoni, Renal Unit, Careggi Hospital, Careggi University, Florence 50139, Italy.

Published: August 2013

AI Article Synopsis

  • * Complement proteins play a significant role in all types of thrombotic microangiopathy, including typical HUS, atypical HUS, and thrombotic thrombocytopenic purpura (TTP), with certain secondary HUS forms linked to complement gene abnormalities.
  • * Treatment options for HUS include traditional therapies like plasma therapy and kidney transplants, alongside emerging treatments such as the monoclonal antibody eculizumab, which shows promise for atypical HUS and potentially for typical HUS and TTP

Article Abstract

Hemolytic uremic syndrome (HUS) is a rare disease. In this work the authors review the recent findings on HUS, considering the different etiologic and pathogenetic classifications. New findings in genetics and, in particular, mutations of genes that encode the complement-regulatory proteins have improved our understanding of atypical HUS. Similarly, the complement proteins are clearly involved in all types of thrombotic microangiopathy: typical HUS, atypical HUS and thrombotic thrombocytopenic purpura (TTP). Furthermore, several secondary HUS appear to be related to abnormalities in complement genes in predisposed patients. The authors highlight the therapeutic aspects of this rare disease, examining both "traditional therapy" (including plasma therapy, kidney and kidney-liver transplantation) and "new therapies". The latter include anti-Shiga-toxin antibodies and anti-C5 monoclonal antibody "eculizumab". Eculizumab has been recently launched for the treatment of the atypical HUS, but it appears to be effective in the treatment of typical HUS and in TTP. Future therapies are in phases I and II. They include anti-C5 antibodies, which are more purified, less immunogenic and absorbed orally and, anti-C3 antibodies, which are more powerful, but potentially less safe. Additionally, infusions of recombinant complement-regulatory proteins are a potential future therapy.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3832913PMC
http://dx.doi.org/10.5527/wjn.v2.i3.56DOI Listing

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