American Journal of Kidney Diseases
Volume 55, Issue 4 , Pages 708-711 , April 2010

Maintenance of Kidney Function Following Treatment With Eculizumab and Discontinuation of Plasma Exchange After a Third Kidney Transplant for Atypical Hemolytic Uremic Syndrome Associated With a CFH Mutation

  • Jean-Claude Davin, MD, PhD

      Affiliations

    • Paediatric Nephrology, Emma Children's Hospital/Academic Medical Centre, Amsterdam, The Netherlands
    • Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium
    • Corresponding Author InformationAddress correspondence to Jean-Claude Davin, MD, PhD, Pediatric Nephrology Department, Emma Children's Hospital/Academic Medical Center of Amsterdam, Meibergdreef, 9, 1105 AZ Amsterdam Zuid-Oost, The Netherlands
    • ,
  • Valentina Gracchi, MD

      Affiliations

    • Paediatric Nephrology, Emma Children's Hospital/Academic Medical Centre, Amsterdam, The Netherlands
    • ,
  • Antonia Bouts, MD, PhD

      Affiliations

    • Paediatric Nephrology, Emma Children's Hospital/Academic Medical Centre, Amsterdam, The Netherlands
    • ,
  • Jaap Groothoff, MD, PhD

      Affiliations

    • Paediatric Nephrology, Emma Children's Hospital/Academic Medical Centre, Amsterdam, The Netherlands
    • ,
  • Lisa Strain, PhD

      Affiliations

    • Institute of Human Genetics, Newcastle University, Newcastle Upon Tyne, United Kingdom
    • ,
  • Tim Goodship, MD, PhD

      Affiliations

    • Institute of Human Genetics, Newcastle University, Newcastle Upon Tyne, United Kingdom

Received 8 April 2009 ,Accepted 6 August 2009.

References 

  1. Bresin E, Daina E, Noris M, et al. Outcome of renal transplantation in patients with non-Shiga toxin-associated hemolytic uremic syndrome: prognostic significance of genetic background. Clin J Am Soc Nephrol. 2006;1:88–99
  2. Olie KH, Goodship THJ, Verlaak R, et al. Post transplantation cytomegalovirus-induced recurrence of atypical haemolytic uremic syndrome associated with a factor H mutation: successful treatment with intensive plasma exchanges and ganciclovir. Am J Kidney Dis. 2005;45:12–15
  3. Davin JC, Olie KH, Verlaak R, et al. Complement factor H-associated atypical hemolytic uremic syndrome in monozygotic twins: concordant presentation, discordant response to treatment. Am J Kidney Dis. 2006;47(2):e27–e30
  4. Davin JC, Strain L, Goodship TH. Plasma therapy in atypical haemolytic uremic syndrome: lessons from a family with a factor H mutation. Pediatr Nephrol. 2008;23:1517–1521
  5. Vergouwen MD, Adriani KS, Roos YB, Groothoff JW, Majoie CB. Proximal cerebral artery stenosis in a patient with hemolytic uremic syndrome. [letter] AJNR Am J Neuroradiol. 2008;29(5):e34
  6. Seelen MA, Roos A, Wieslander J, et al. Functional analysis of the classical, alternative, and MBL pathways of the complement system: standardization and validation of a simple ELISA. J Immunol Methods. 2005;296(1-2):187–198
  7. Sanchez-Corral P, Perez-Caballero D, Huarte O, et al. Structural and functional characterization of factor H mutations associated with atypical hemolytic uremic syndrome. Am J Hum Genet. 2002;71:1285–1295
  8. Richards A, Buddles MR, Donne RL, et al. Factor H mutations in hemolytic uremic syndrome cluster in exons 18–20, a domain important for host cell recognition. Am J Hum Genet. 2001;68:485–490
  9. Manuelian T, Hellwage J, Meri S, et al. Mutations in factor H reduce binding affinity to C3b and heparin and surface attachment to endothelial cells in hemolytic uremic syndrome. J Clin Invest. 2003;111:1181–1190
  10. Saland JM, Emre SH, Shneider BL, et al. Favorable long-term outcome after liver-kidney transplant for recurrent hemolytic uremic syndrome associated with a factor H mutation. Am J Transplant. 2006;6:1948–1952
  11. Jalanko H, Peltonen S, Koskinen A, et al. Successful liver-kidney transplantation in two children with aHUS caused by a mutation in complement factor H. Am J Transplant. 2008;8:216–221
  12. Saland JM, Shneider BL, Bromberg JS, et al. Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome. Clin J Am Soc Nephrol. 2009;4(1):201–206
  13. Saland JM, Ruggenenti P, Remuzzi G Consensus Study Group. Liver-kidney transplantation to cure atypical hemolytic uremic syndrome. J Am Soc Nephrol. 2009;20(5):940–949
  14. Jamieson NV. A 20-year experience of combined liver/kidney transplantation for primary hyperoxaluria (PH1): the European PH1 transplant registry experience 1984-2004. Am J Nephrol. 2005;25:282–289
  15. Gruppo RA, Rother RP. Eculizumab for congenital atypical hemolytic-uremic syndrome. N Engl J Med. 2009;360:544–546
  16. Nurnberger J, Witzke O, Opazo Saez A, et al. Eculizumab for atypical hemolytic-uremic syndrome. N Engl J Med. 2009;360:542–544
  17. Parker C. Eculizumab for paroxysmal nocturnal haemoglobinuria. Lancet. 2009;373:759–767
  18. Brodsky RA, Young NS, Antonioli E, et al. Multicenter phase 3 study of the complement inhibitor eculizumab for the treatment of patients with paroxysmal nocturnal hemoglobinuria. Blood. 2008;15:1840–1847111(4)

 Originally published online as doi:10.1053/j.ajkd.2009.08.011 on October 26, 2009.

PII: S0272-6386(09)01143-3

doi: 10.1053/j.ajkd.2009.08.011

American Journal of Kidney Diseases
Volume 55, Issue 4 , Pages 708-711 , April 2010

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