First-line therapy in atypical hemolytic uremic syndrome: consideration on infants with a poor prognosis.

ABSTRACT:

Background

Atypical hemolytic uremic syndrome (aHUS) is a rare and heterogeneous disorder. The first line treatment of aHUS is plasma therapy, but in the past few years, the recommendations have changed greatly with the advent of eculizumab, a humanized monoclonal anti C5-antibody. Although recent recommendations suggest using it as a primary treatment for aHUS, important questions have arisen about the necessity of immediate use of eculizumab in all cases. We aimed to draw attention to a specific subgroup of aHUS patients with rapid disease progression and high mortality, in whom plasma therapy may not be feasible.

Methods

We present three pediatric patients of acute complement-mediated HUS with a fatal outcome. Classical and alternative complement pathway activity, levels

SUBMITTER: Szarvas N 

PROVIDER: S-EPMC4295478 | biostudies-literature | 2014 Dec

REPOSITORIES: biostudies-literature