Pathophysiology of thrombotic microangiopathies

M. Noris, J. Caprioli, G. Remuzzi

Research output: Contribution to journalArticle

Abstract

Hemolytic uremic syndrome (HUS) and the related condition, thrombotic thrombocytopenic purpura (TTP), are diseases of microangiopathic hemolytic anemia and thrombocytopenia with signs of renal and brain involvement. They are collectively called thrombotic microangiopathies (TMA). The classical, diarrhea-associated form of HUS, that mainly affects infants and has a good outcome. In contrast to children, adults with TMA have a higher mortality rate. Injury to the endothelial cells is the key factor in the sequence of events leading to TMA. Intrinsic abnormalities of the complement system and of the von Willebrand Factor pathway may play a crucial role particularly in familial and recurrent forms.

Original languageEnglish
Pages (from-to)37-43
Number of pages7
JournalInternal Medicine Clinical and Laboratory
Volume8
Issue number3
Publication statusPublished - 2000

Keywords

  • Complement
  • Endothelial cells
  • Factor H
  • Hemolytic uremic syndrome
  • Kidney
  • Shiga-like toxins
  • Thrombotic thrombocytopenic purpura
  • Von Willebrand Factor

ASJC Scopus subject areas

  • Internal Medicine

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