Abstract
Hemolytic uremic syndrome caused by Shiga toxin-producing E. coli (STEC-HUS) is often associated with a severe morbidity including neurological involvement and a mortality of 1-5%. Although STEC-HUS is often self-limited, improvement of treatment strategies is needed for cases with complications and, among others, plasma exchange/plasmapheresis and use of antibiotics have been advocated. With the availability of the complement blocker eculizumab, now a standard treatment of atypical HUS, several series have addressed its use in STEC-HUS, with variable response; randomized controlled trials are lacking.In this issue of Pediatric Nephrology, Pecheron et al. present a cohort of 33 pediatric patients with severe HUS treated with eculizumab. Neurological involvement was observed in 85% of the patients and 94% required dialysis. Most patients (55%) did not benefit from eculizumab and renal dysfunction as well as neurological sequelae did not resolve. In a subgroup of patients, however, rapid neurological improvement was described. In the post-hoc-defined group of patients with favorable outcome, there was a trend towards more sustained complement inhibition, although this finding was not significant compared to patients with an unfav...Continue Reading
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