Transplantation in atypical hemolytic uremic syndrome.
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.
Kavanagh
David
D
Institute of Human Genetics, Newcastle University, United Kingdom. davidkavanagh@doctors.org.uk
Richards
Anna
A
Goodship
Tim
T
Jalanko
Hannu
H
D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D003165:Complement System Proteins / Q000235:genetics / Q000276:immunology; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D006801:Humans; D016030:Kidney Transplantation; D016031:Liver Transplantation; D009154:Mutation; D010951:Plasma Exchange
D000911:Antibodies, Monoclonal; D003165:Complement System Proteins