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Long‐term remission with eculizumab in atypical haemolytic uraemic syndrome
Author(s) -
Carter Simon,
Hewitt Ian,
Kausman Joshua
Publication year - 2017
Publication title -
nephrology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.752
H-Index - 61
eISSN - 1440-1797
pISSN - 1320-5358
DOI - 10.1111/nep.12932
Subject(s) - eculizumab , atypical hemolytic uremic syndrome , medicine , haemolytic uraemic syndrome , complement system , adverse effect , monoclonal , renal function , immunology , complement (music) , monoclonal antibody , pediatrics , antibody , genetics , escherichia coli , complementation , biology , gene , phenotype
The understanding of the role of complement dysregulation in atypical haemolytic uraemic syndrome (aHUS) has led to major changes in therapeutic approaches and outcomes. Eculizumab is a humanized anti‐C5 monoclonal antibody that inhibits the terminal complement pathway and has revolutionized the treatment and prognosis of aHUS. However, published reports to date have had relatively short‐term follow‐up. We report two paediatric cases of aHUS successfully treated with eculizumab longer than 6 years with no serious adverse events and preservation of renal function.