Literature DB >> 23461281

Changing strategies for organ transplantation in atypical haemolytic uraemic syndrome: a tertiary case series.

Thomas A Forbes1, Mark G Bradbury, Tim H J Goodship, Patrick J McKiernan, David V Milford.   

Abstract

We present three cases of organ transplantation for atypical haemolytic uraemic syndrome secondary to complement factor H mutation: one isolated renal transplant; one previously reported isolated liver transplant; and one combined liver and kidney transplant. All three patients were treated prior to the licensing of eculizumab for this condition, and all have had favourable outcomes with maintenance of graft function for years following transplantation. We discuss the evolution of transplantation therapy for aHUS over the last two decades. Transplantation decision-making in aHUS has evolved over this time with expanding knowledge of pathophysiology and genetics, alongside refined plasma exchange and anticoagulation protocols and improved centre experience. Our cases demonstrate how individual patient factors within this heterogeneous condition also underlie transplantation decisions and outcomes. Whilst our cases demonstrate that transplantation in aHUS can be a successful long-term treatment providing good quality of life, worldwide experience has proven that most curative treatment for aHUS strategies represents significant risks. Whether new pharmacotherapies such as eculizumab will alter this risk is yet to be determined.
© 2013 John Wiley & Sons A/S.

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Year:  2013        PMID: 23461281     DOI: 10.1111/petr.12066

Source DB:  PubMed          Journal:  Pediatr Transplant        ISSN: 1397-3142


  6 in total

Review 1.  An international consensus approach to the management of atypical hemolytic uremic syndrome in children.

Authors:  Chantal Loirat; Fadi Fakhouri; Gema Ariceta; Nesrin Besbas; Martin Bitzan; Anna Bjerre; Rosanna Coppo; Francesco Emma; Sally Johnson; Diana Karpman; Daniel Landau; Craig B Langman; Anne-Laure Lapeyraque; Christoph Licht; Carla Nester; Carmine Pecoraro; Magdalena Riedl; Nicole C A J van de Kar; Johan Van de Walle; Marina Vivarelli; Véronique Frémeaux-Bacchi
Journal:  Pediatr Nephrol       Date:  2015-04-11       Impact factor: 3.714

Review 2.  Hemolytic uremic syndrome.

Authors:  Caterina Mele; Giuseppe Remuzzi; Marina Noris
Journal:  Semin Immunopathol       Date:  2014-02-14       Impact factor: 9.623

3.  Liver-kidney transplantation to cure atypical HUS: still an option post-eculizumab?

Authors:  Jeffrey Saland
Journal:  Pediatr Nephrol       Date:  2013-12-22       Impact factor: 3.714

4.  Liver transplantation for aHUS: still needed in the eculizumab era?

Authors:  Rosanna Coppo; Roberto Bonaudo; R Licia Peruzzi; Alessandro Amore; Andrea Brunati; Renato Romagnoli; Mauro Salizzoni; Miriam Galbusera; Eliana Gotti; Erica Daina; Marina Noris; Giuseppe Remuzzi
Journal:  Pediatr Nephrol       Date:  2015-11-24       Impact factor: 3.714

5.  Successful eculizumab treatment of recurrent postpartum atypical hemolytic uremic syndrome after kidney transplantation.

Authors:  Katherine Garlo; Doug Dressel; Marizela Savic; John Vella
Journal:  Clin Nephrol Case Stud       Date:  2015-06-15

6.  Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea.

Authors:  Vicky Brocklebank; David Kavanagh
Journal:  Clin Kidney J       Date:  2017-05-08
  6 in total

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