Clinical practice guidelines for the management of atypical haemolytic uraemic syndrome in the United Kingdom.
Atypical haemolytic uraemic syndrome (aHUS) is associated with a poor prognosis with regard to survival at presentation, recovery of renal function and transplantation. It is now established that aHUS is a disease of complement dysregulation with mutations in the genes encoding both complement regulators and activators, and autoantibodies against the complement regulator factor H. Identification of the underlying molecular abnormality in an individual patient can now help to guide their future management. In these guidelines we make recommendations for the investigation and management of aHUS patients both at presentation and in the long-term. We particularly address the role of renal transplantation alone and combined liver-kidney transplantation.
Taylor
C Mark
CM
Institute of Human Genetics, Newcastle University, Central Parkway, Newcastle upon Tyne, UK.
Machin
Sam
S
Wigmore
Stephen J
SJ
Goodship
Tim H J
TH
D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D002986:Clinical Trials as Topic; D006463:Hemolytic-Uremic Syndrome / Q000145:classification / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D016030:Kidney Transplantation; D016031:Liver Transplantation; D010951:Plasma Exchange / Q000379:methods; D009626:Terminology as Topic
D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab