Shiga toxin associated hemolytic uremic syndrome

Hematol Oncol Clin North Am. 2015 Jun;29(3):525-39. doi: 10.1016/j.hoc.2015.01.007.

Abstract

Shiga toxin associated hemolytic uremic syndrome (Stx HUS), a thrombotic microangiopathy, is the most common cause of pediatric acute kidney injury but has no direct treatment. A better understanding of disease pathogenesis may help identify new therapeutic targets. For this reason, the role of complement is being actively studied while eculizumab, the C5 monoclonal antibody, is being used to treat Stx HUS but with conflicting results. A randomized controlled trial would help properly evaluate its use in Stx HUS while more research is required to fully evaluate the role complement plays in the disease pathogenesis.

Keywords: Complement; Eculizumab; Shiga toxin; Thrombotic microangiopathy.

Publication types

  • Review

MeSH terms

  • Acute Kidney Injury / etiology
  • Acute Kidney Injury / immunology*
  • Child
  • Complement Pathway, Alternative / immunology
  • Complement System Proteins / immunology
  • Complement System Proteins / metabolism
  • Hemolytic-Uremic Syndrome / complications
  • Hemolytic-Uremic Syndrome / immunology*
  • Hemolytic-Uremic Syndrome / microbiology
  • Humans
  • Models, Immunological
  • Shiga Toxin / immunology*
  • Shiga Toxin / metabolism
  • Shiga-Toxigenic Escherichia coli / immunology*
  • Shiga-Toxigenic Escherichia coli / metabolism
  • Shiga-Toxigenic Escherichia coli / physiology
  • Thrombotic Microangiopathies / complications
  • Thrombotic Microangiopathies / immunology*

Substances

  • Shiga Toxin
  • Complement System Proteins