FH-aHUS hemolytic agarose plate: A descendents of Sanchez-corral assay for a rapid diagnosis of hereditary and acquired factor H-related Atypical Hemolytic Uremic Syndrome

Abstract

INTRODUCTION Hemolytic uremic syndrome (HUS) is not only known by its approved drug Eculizumab, the most expensive medicine up to now (An annual drug cost US $573,720 per patient), but also by its catastrophic outcomes (70% of patients progress to death or renal failure in less than 1 year after the onset). In the absence of repetitive infections, this ultra-rare form of thrombotic microangiopathy (TMA) is qualified by the “atypical” form of HUS (aHUS). The factor H related- aHUS (fH-aHUS) belongs of this latter and represents more than a half of the aHUS. It is caused by genetic or acquired autoimmune defects of the alternative complement pathway regulation by the factor H.