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World J Nephrol. Aug 6, 2013; 2(3): 56-76
Published online Aug 6, 2013. doi: 10.5527/wjn.v2.i3.56
Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations
Maurizio Salvadori, Elisabetta Bertoni
Maurizio Salvadori, Elisabetta Bertoni, Renal Unit, Careggi Hospital, Careggi University, Florence 50139, Italy
Author contributions: Salvadori M and Bertoni E equally contributed to writing the paper, collecting the literature data, revising both the scientific contents and the English wording, and gave the final approval of the version to be published.
Correspondence to: Maurizio Salvadori, MD, Professor, Renal Unit, Careggi Hospital, Careggi University, Viale Pieraccini 18, Florence 50139, Italy.
Telephone: +39-55-7949269 Fax: +39-55-435878
Received: May 7, 2013
Revised: June 26, 2013
Accepted: August 2, 2013
Published online: August 6, 2013
Core Tip

Core tip: Hemolytic uremic syndrome (HUS) is a rare disease, knowledge of which is rapidly increasing. The disease takes several forms, but recent data suggest that the physiopathologic basis in the vast majority of such diseases is complement dysregulation. New therapies are available, including a monoclonal antibody that blocks the C5 cascade. Such therapies lead to a substantial improvement in the outcome, which previously was often poor. HUS often occurs secondary to other diseases. In such cases, diagnosis may be difficult due to the overlapping of two diseases. Complement dysregulation has been found also in secondary HUS.