Complement disorders and hemolytic uremic syndrome

Catherine Joseph, Jyothsna Gattineni

Research output: Contribution to journalReview article

19 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)209-215
Number of pages7
JournalCurrent opinion in pediatrics
Volume25
Issue number2
DOIs
StatePublished - Apr 2013

Keywords

  • Factor H
  • Factor I
  • Membrane cofactor protein
  • Plasma exchange

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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