title: Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome.
abstract: Haemolytic-uraemic syndrome is a clinical syndrome characterized by thrombocytopaenia, non-autoimmune haemolytic anaemia and renal impairment. Pathological alterations in kidney samples show thrombotic microangiopathy. The underlying pathogenesis is endothelial cell injury with thrombotic occlusion of the arterioles and capillaries. A variety of causes have been identified, associated with infection of Escherichia coli O157:H7, environmental factors as immunosuppressive drugs and genetic deficiencies in complement regulatory factors. The latter is called atypical haemolytic-uraemic syndrome (aHUS). Here, we present a patient with severe aHUS with complement factor H deficiency triggered by cocaine use and recurrence after kidney transplantation. The patient restarted haemodialysis for severe renal insufficiency and anti-C5 antibody eculizumab was used as salvage treatment with progressive recovery of graft function and suppression of dialysis.
authors.affiliation: Nephrology and Urology Clinical Institute (ICNU), Hospital Clinic, University of Barcelona, Barcelona, Spain and Hospital Clinic, Institut d'investigacions Biomèdiques August Pi i Suñer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Durán
authors.affiliation: Nephrology and Urology Clinical Institute (ICNU), Hospital Clinic, University of Barcelona, Barcelona, Spain and Hospital Clinic, Institut d'investigacions Biomèdiques August Pi i Suñer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Miquel
authors.initials: M
authors.lastname: Blasco
authors.affiliation: Nephrology and Urology Clinical Institute (ICNU), Hospital Clinic, University of Barcelona, Barcelona, Spain and Hospital Clinic, Institut d'investigacions Biomèdiques August Pi i Suñer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Francisco
authors.initials: F
authors.lastname: Maduell
authors.affiliation: Nephrology and Urology Clinical Institute (ICNU), Hospital Clinic, University of Barcelona, Barcelona, Spain and Hospital Clinic, Institut d'investigacions Biomèdiques August Pi i Suñer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Josep M
authors.initials: JM
authors.lastname: Campistol
keywords: atypical haemolytic-uraemic syndrome; kidney transplantation; monoclonal antibody