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Use of eculizumab in atypical uremic hemolytic syndrome in childhood: case report


Urology & Nephrology Open Access Journal
Mariana Marta de Oliveira Antunes, Maria Goretti Moreira Guimarães Penido

Abstract

Hemolytic-uremic syndrome is a form of thrombotic microangiopathy and is currently classified in childhood as typical and atypical. The atypical hemolytic-uremic syndrome encompasses genetic mutations in factors that regulate the complement cascade or antibodies against factor H. The typical form is caused by Shiga-toxin-producing Escherichia coli. There is also secondary urethral hemolytic syndrome due to an infectious condition such as pneumonia by S. pneumoniae, autoimmune diseases and use of medications with calcineurin inhibitors. The best knowledge of the pathophysiology of atypical hemolyticuremic syndrome allowed the use of Eculizumab, a monoclonal antibody that acts in the final phase of the complement cascade. The objective of this study was to report a case of a patient with atypical hemolytic-uremic syndrome using Eculizumab, who was suspended for lack of medication and presented recurrence of the disease.

Keywords

atypical hemolytic-uremic syndrome, thrombotic microangiopathy, eculizumab

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