Complement disorders and hemolytic uremic syndrome

Curr Opin Pediatr. 2013 Apr;25(2):209-15. doi: 10.1097/MOP.0b013e32835df48a.

Abstract

Purpose of review: 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.

Recent findings: In the last decade, strides have been made in identifying several new disease-causing mutations in complement-regulating proteins.

Summary: 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.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Atypical Hemolytic Uremic Syndrome
  • Complement Activation / immunology
  • Complement System Proteins / genetics
  • Complement System Proteins / immunology*
  • Hemolytic-Uremic Syndrome / genetics
  • Hemolytic-Uremic Syndrome / immunology*
  • Hemolytic-Uremic Syndrome / therapy
  • Humans
  • Kidney Failure, Chronic / immunology
  • Kidney Transplantation
  • Plasma Exchange / methods
  • Prognosis
  • Recurrence

Substances

  • Complement System Proteins