Miroslav Šašinka, Katarína Furková
Over the past decade, new insights into the role of complement in the pathogenesis of atypical hemolytic-uremic syndrome (aHUS) have resulted in a better understanding of the natural history of the disease and opened the way to new therapies. Basic pathogenic mechanism in this disease is uncontrolled alternative way complement activation. There were characterized many new and complex genetic disorders namely mutations and polymorphisms associated with this activation and aHUS. Until recently, plasma therapy (infusions or plasmapheresis) was the only treatment _option. In most patients, however, plasma therapy proved ineffective at preventing long term-consequences of disease, with most patients progressing to end stage renal failure or death within 1-3 years. Kidney transplantation often ended in the graft loss. The arrival of monoclonal antibody to C5a protein eculizumab as an approved new form of therapy for aHUS changed the standard care for this disease. For pediatricians its means to know early diagnostics of aHUS and early starting with treatment.