Summary of Westra, D. et al; Serological and genetic complement alterations in infection-induced and complement-mediated hemolytic uremic syndrome. Pediatric Nephrology 2017, 32(2). Click here for the full article.

The role of complement in the atypical form of hemolytic uremic syndrome (aHUS) has been extensively studied. However, less is known about the role of complement in infection-induced HUS. In a majority of these cases, the disease is triggered by an infection with Shiga-like toxin-producing Escherichia coli (STEC-HUS). Based on the similarity of the clinical manifestations of complement-mediated and infection-induced HUS, this study hypothesizes that also here the dysregulation of the complement system has an important place in the pathogenesis.

To determine the role of the alternative complement pathway (AP), 37 pediatric patients were enrolled in this study. 26 patients had STEC-HUS and 11 were diagnosed with aHUS. EDTA plasma was taken from the patients and used for testing a variety of complement factors and components, such as C3, CFH, CFI and TCC (Cat. # HK328). Results show that TCC was only significantly increased in STEC-HUS patients in the acute phase […] making it useful in monitoring the course of the disease. Taken all aspects together it is clear that measuring AP activation products, in particular C3d/C3 ratio, might help in monitoring disease activity and distinguishing between the different HUS etiologies. The exact role of altered complement activation in the pathogenesis of STEC-HUS, however, has not been fully elucidated.