title: Eculizumab in atypical haemolytic-uraemic syndrome allows cessation of plasma exchange and dialysis.
abstract: Disorders in complement regulation are a major cause of atypical haemolytic-uraemic syndrome (aHUS). Eculizumab, a monoclonal antibody targeting complement C5 and blocking the terminal complement cascade, should theoretically be useful in this disease, particularly when associated with specific complement pathway anomalies such as Factor H deficiency. Eculizumab is emerging as an effective treatment for post-transplant aHUS recurrence and may have a role in treating de novo aHUS, halting the haemolytic process. In this case report, we describe the fourth case of aHUS treated with eculizumab. In our patient, with a known complement Factor H mutation, not only has the disease process become quiescent but also this therapy has led to significantly improved renal function so that dialysis is no longer necessary.
authors.affiliation: Department of Paediatric Nephrology, Evelina Children's Hospital, London, UK.
authors.forename: Jon Jin
authors.initials: JJ
authors.lastname: Kim
authors.affiliation: Department of Paediatric Nephrology, Evelina Children's Hospital, London, UK.
authors.forename: Simon C
authors.initials: SC
authors.lastname: Waller
authors.affiliation: Department of Paediatric Nephrology, Evelina Children's Hospital, London, UK.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Reid
keywords: atypical haemolytic–uraemic syndrome; dialysis; eculizumab; plasma exchange