Advances in our understanding of the pathogenesis of hemolytic uremic syndromes

Am J Physiol Renal Physiol. 2018 Mar 1;314(3):F454-F461. doi: 10.1152/ajprenal.00376.2017. Epub 2017 Nov 22.


Hemolytic uremic syndrome (HUS) is major global health care issue as it is the leading cause of acute kidney injury in children. It is a triad of acute kidney injury, microangiopathic hemolytic anemia, and thrombocytopenia. In recent years, major advances in our understanding of complement-driven inherited rare forms of HUS have been achieved. However, in children 90% of cases of HUS are associated with a Shiga toxin-producing enteric pathogen. The precise pathological mechanisms in this setting are yet to be elucidated. The purpose of this review is to discuss advances in our understanding of the pathophysiology underlying HUS and identify the key questions yet to be answered by the scientific community.

Keywords: complement; hemolytic uremic syndrome; thrombotic microangiopathy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Acute Kidney Injury / etiology*
  • Acute Kidney Injury / genetics
  • Acute Kidney Injury / immunology
  • Acute Kidney Injury / microbiology
  • Animals
  • Atypical Hemolytic Uremic Syndrome / etiology*
  • Atypical Hemolytic Uremic Syndrome / genetics
  • Atypical Hemolytic Uremic Syndrome / immunology
  • Atypical Hemolytic Uremic Syndrome / microbiology
  • Complement Activation* / genetics
  • Complement System Proteins / genetics
  • Complement System Proteins / immunology*
  • Escherichia coli Infections / microbiology*
  • Genetic Predisposition to Disease
  • Humans
  • Phenotype
  • Prognosis
  • Risk Factors
  • Shiga-Toxigenic Escherichia coli / pathogenicity*
  • Thrombotic Microangiopathies / etiology*
  • Thrombotic Microangiopathies / genetics
  • Thrombotic Microangiopathies / immunology
  • Thrombotic Microangiopathies / microbiology


  • Complement System Proteins