title: Complement disorders and hemolytic uremic syndrome.
abstract: OBJECTIVE
  Complement mediated hemolytic uremic syndrome (aHUS) accounts for a significant proportion of non-shiga toxin HUS. The purpose of this review is to outline the pathophysiology, clinical features and therapeutic options for aHUS.
  
  
  RESULTS
  In the last decade, strides have been made in identifying several new disease-causing mutations in complement-regulating proteins.
  
  
  CONCLUSIONS
  Complement mediated HUS (aHUS) has a worse prognosis compared with shiga toxin mediated HUS, often resulting in end stage renal disease. Early identification of aHUS is crucial so that plasma therapy can be initiated. After renal transplantation, there is very high risk of disease recurrence and graft loss. Eculizumab and combined liver-kidney transplantation offer promise for improved prognosis.
authors: Department of Pediatrics, University of Texas, Southwestern Medical Center, Dallas, Texas 75390-9063, USA.
authors: Catherine
authors: C
authors: Joseph
authors: Jyothsna
authors: J
authors: Gattineni
mesh_terms: D065766:Atypical Hemolytic Uremic Syndrome; D003167:Complement Activation / Q000276:immunology; D003165:Complement System Proteins / Q000235:genetics / Q000276:immunology*; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics / Q000276:immunology* / Q000628:therapy; D006801:Humans; D007676:Kidney Failure, Chronic / Q000276:immunology; D016030:Kidney Transplantation; D010951:Plasma Exchange / Q000379:methods; D011379:Prognosis; D012008:Recurrence
chemical_list: D003165:Complement System Proteins
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