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Semin Thromb Hemost 2010; 36(6): 575-585
DOI: 10.1055/s-0030-1262879
© Thieme Medical Publishers

Pathophysiology of Typical Hemolytic Uremic Syndrome

Diana Karpman1 , Lisa Sartz1 , Sally Johnson2
  • 1Department of Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden
  • 2Department of Paediatric Nephrology, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, United Kingdom
Further Information

Publication History

Publication Date:
23 September 2010 (online)

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ABSTRACT

The typical form of hemolytic uremic syndrome (HUS) is associated with enterohemorrhagic Escherichia coli (EHEC) infection. The disease process is initiated and perpetuated by interactions between the pathogen or its virulence factors and host cells, as well as the host response. During EHEC-associated HUS, alterations occurring at the intestinal mucosal barrier and in the circulation, as well as on endothelial cells and other target-organ cells, lead to cell activation and/or cytotoxicity, and trigger a prothrombotic state. This review summarizes current knowledge regarding the interactions of the pathogen and its virulence factors with cells in the intestine, bloodstream, kidney, and brain. Mechanisms of bacterial colonization, toxin circulation, and induction of target organ damage are discussed.

KEYWORDS

Hemolytic uremic syndrome - Shiga toxin - enterohemorrhagic Escherichia coli - thrombotic microangiopathy - kidney

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Diana KarpmanM.D. Ph.D. 

Department of Pediatrics, Clinical Sciences Lund

Lund University, Lund, Sweden

Email: diana.karpman@med.lu.se