title: Haemolytic uraemic syndrome caused by factor H mutation: is single kidney transplantation under intensive plasmatherapy an option?
abstract: Complement factor H (CFH) mutation is one of the causes of atypical haemolytic uraemic syndrome (aHUS). Patients with CFH mutation-associated aHUS progress often to end-stage renal disease despite plasma exchange therapy. When such patients are transplanted, aHUS recurs almost invariably and causes graft failure making the rationale of single kidney allograft transplantation questionable. Since CFH is synthesized mostly by the liver, combined liver-kidney transplantation has been recommended. However, fatal outcomes have been reported using this strategy. We report a case of successful single kidney allograft transplantation in a patient with a CFH gene mutation (R1210C), who had end-stage renal failure after three flares of aHUS treated with plasma exchange. He received peri- and postoperative infusions of fresh frozen plasma, which to date has prevented recurrence of the disease. He has preserved renal function 1-year post-transplant.
authors.affiliation: Division of Transplantation Immunology and Nephrology, University Hospital Basel, Basel, Switzerland. phirt@uhbs.ch
authors.forename: Patricia
authors.initials: P
authors.lastname: Hirt-Minkowski
authors.forename: Stefan
authors.initials: S
authors.lastname: Schaub
authors.forename: Michael
authors.initials: M
authors.lastname: Mayr
authors.forename: Jürg A
authors.initials: JA
authors.lastname: Schifferli
authors.forename: Michael
authors.initials: M
authors.lastname: Dickenmann
authors.forename: Véronique
authors.initials: V
authors.lastname: Frémeaux-Bacchi
authors.forename: Jürg
authors.initials: J
authors.lastname: Steiger
mesh_terms: D000328:Adult; D017242:Complement Factor H / Q000235:genetics*; D006463:Hemolytic-Uremic Syndrome / Q000209:etiology / Q000235:genetics / Q000628:therapy*; D006801:Humans; D016030:Kidney Transplantation; D008297:Male; D009154:Mutation; D010951:Plasma Exchange
chemical_list: D017242:Complement Factor H