Conclusions
Therapy of recurrent/aHUS is not evidence based. However, several reports in the literature and the personal experience of the authors suggests that many patients with complement disorders respond well to any form of plasma therapy. Most centers prefer plasma exchange in the beginning and continue with plasma infusion as mainstay or prophylaxis. Despite this active and vigorous regimen many patients succumb to end-stage renal disease. Transplantation of either kidney alone or combined kidney/liver transplantation bear a high risk of recurrence. New therapeutic strategies are therefore absolutely mandatory. Hopefully, new insights in the mechanisms of disease and perhaps new complement inhibitors (such as humanized anti C5 antibodies) may improve treatment and outcome of these poor patients.
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Würzner, R., Zimmerhackl, L.B. (2006). Therapeutic strategies for atypical and recurrent hemolytic uremic syndromes (HUS). In: Zipfel, P.F. (eds) Complement and Kidney Disease. Progress in Inflammation Research. Birkhäuser Basel. https://doi.org/10.1007/3-7643-7428-4_9
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