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Semin Thromb Hemost 2010; 36(6): 653-659
DOI: 10.1055/s-0030-1262887
© Thieme Medical Publishers

Transplantation in Atypical Hemolytic Uremic Syndrome

David Kavanagh1 , Anna Richards2 , Tim Goodship1 , Hannu Jalanko3
  • 1Institute of Human Genetics, Newcastle University, United Kingdom
  • 2MRC Centre for Inflammation Research, Queens Medical Research Institute, Edinburgh, United Kingdom
  • 3Hospital for Children and Adolescents, Helsinki University Central Hospital, Helsinki, Finland
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Publication History

Publication Date:
23 September 2010 (online)

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ABSTRACT

Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by overactivation of complement. Recurrence following renal transplantation is determined by a genetic predisposition. Genetic screening of all individuals with aHUS should be performed prior to listing for transplantation. Individuals with isolated mutations in MCP have a low risk of recurrence and may be considered for kidney transplantation alone. In individuals with CFH and CFI mutations, the risk of recurrence following renal transplantation is high. Combined liver/kidney transplantation has been used successfully in individuals with CFH mutations following the introduction of perioperative plasma exchange; however, such a procedure is not without its risks. Liver/kidney transplantation has yet to be performed on individuals with CFI and C3 mutations but may be predicted to be successful. In individuals with CFH autoantibodies, a reduction in titer through plasma exchange and rituximab has been successful. Clinical trials of the complement C5 inhibitor eculizumab may improve prospects for isolated renal transplantation in individuals with complement protein mutations.

KEYWORDS

Transplantation - hemolytic uremic syndrome - complement - factor H - eculizumab

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David Kavanagh

Institute of Human Genetics, International Centre for Life, Central Parkway

Newcastle upon Tyne, NE1 3BZ United Kingdom

Email: davidkavanagh@doctors.org.uk