title: Living kidney transplantation in adult patients with atypical haemolytic uraemic syndrome.
abstract: BACKGROUND
  Dysregulation of complement activation is the most common cause of the atypical haemolytic uraemic syndrome (aHUS). Many patients with aHUS develop end-stage renal disease and consider kidney transplantation. However, the recurrence rate after transplantation ranges from 45-90% in patients with known abnormalities in circulating complement proteins. It was recently proposed that patients with aHUS should be treated prophylactically with plasma exchange or eculizumab to prevent recurrence after transplantation.
  
  
  METHODS
  A case series describing the successful outcome of kidney transplantation without prophylactic therapy in four adult patients with aHUS and a high risk of disease recurrence. Patients received a living donor kidney and immunosuppression consisting of basiliximab induction, low-dose tacrolimus, prednisone and mycophenolate mofetil. Patients received a statin, and were targeted to a low blood pressure preferably using blockers of the renin-angiotensin system.
  
  
  RESULTS
  After a follow-up of 16-21 months, none of the patients developed recurrent aHUS. Also, no rejection was observed.
  
  
  CONCLUSIONS
  Kidney transplantation in adult patients with aHUS can be successful without prophylactic eculizumab, using a protocol that minimises cold ischaemia time, reduces the risk of rejection and provides endothelial protection. Our data suggest that in patients with aHUS, controlled trials are needed to demonstrate the optimal strategy.
authors.affiliation: Department of Nephrology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands.
authors.forename: J C
authors.initials: JC
authors.lastname: Verhave
authors.forename: D
authors.initials: D
authors.lastname: Westra
authors.forename: H W
authors.initials: HW
authors.lastname: van Hamersvelt
authors.forename: M
authors.initials: M
authors.lastname: van Helden
authors.forename: N C A J
authors.initials: NC
authors.lastname: van de Kar
authors.forename: J F M
authors.initials: JF
authors.lastname: Wetzels
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D065766:Atypical Hemolytic Uremic Syndrome; D000077552:Basiliximab; D050377:Cold Ischemia; D004359:Drug Therapy, Combination; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000150:complications* / Q000235:genetics / Q000517:prevention & control*; D006801:Humans; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000601:surgery; D016030:Kidney Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D011993:Recombinant Fusion Proteins / Q000627:therapeutic use; D055502:Secondary Prevention; D016559:Tacrolimus / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D007166:Immunosuppressive Agents; D011993:Recombinant Fusion Proteins; D000077552:Basiliximab; C481642:eculizumab; D009173:Mycophenolic Acid; D011241:Prednisone; D016559:Tacrolimus