Novel aspects of atypical haemolytic uraemic syndrome and the role of eculizumab

摘要: The haemolytic uraemic syndrome (HUS) is part of a spectrum thrombotic microangiopathies. most common etiologies HUS are the ones seen in childhood caused by an infection Shiga toxin-producing Escherichia coli, with Streptococcus pneumoniae and due to abnormalities alternative pathway complement system. In past decade, enormous progress has been made understanding pathogenesis latter group patients. analysis genes that encode for regulatory proteins development assays measuring activity ADAMTS13 detection antibodies against factor H contributed significantly diagnostic tools patients HUS. These have it possible clearly differentiate between thrombocytopenic purpura various forms With introduction eculizumab, monoclonal anti-C5 inhibitor, clinical arena as effective treatment complement-mediated HUS, new era begun. We review recent advances focus on treatment. discuss unsolved questions, which should be addressed future studies.