Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome

Pathol Biol (Paris). 2015 Jun;63(3):136-43. doi: 10.1016/j.patbio.2015.03.001. Epub 2015 Apr 3.

Abstract

Hemolytic uremic syndrome is a rare disease, frequently responsible for renal insufficiency in children. Recent findings have led to renewed interest in this pathology. The discovery of new gene mutations in the atypical form of HUS and the experimental data suggesting the involvement of the complement pathway in the typical form, open new perspectives for treatment. This review summarizes the current state of knowledge on both typical and atypical hemolytic uremic syndrome pathophysiology and examines new perspectives for treatment.

Keywords: Analogue des récepteurs aux Shiga toxines; Eculizumab; Hemolytic uremic syndrome; Shiga toxin receptor analogues; Syndrome hémolytique urémique; Urtoxazumab.

Publication types

  • Review

MeSH terms

  • Animals
  • Anti-Bacterial Agents / therapeutic use
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Bacterial Infections / complications
  • Bacterial Toxins / adverse effects
  • Clinical Trials as Topic
  • Complement System Proteins / physiology
  • Disease Models, Animal
  • Drug Evaluation, Preclinical
  • Escherichia coli Infections / complications
  • Escherichia coli Infections / microbiology
  • Forecasting
  • Genetic Predisposition to Disease
  • Hemolytic-Uremic Syndrome / classification
  • Hemolytic-Uremic Syndrome / etiology
  • Hemolytic-Uremic Syndrome / genetics
  • Hemolytic-Uremic Syndrome / microbiology
  • Hemolytic-Uremic Syndrome / physiopathology*
  • Hemolytic-Uremic Syndrome / therapy
  • Humans
  • Kidney Transplantation
  • Liver Transplantation
  • Mice
  • Papio
  • Plasma
  • Plasma Substitutes
  • Shiga Toxin / adverse effects
  • Shiga-Toxigenic Escherichia coli / immunology
  • Shiga-Toxigenic Escherichia coli / pathogenicity
  • Thrombophilia / etiology
  • Vascular Endothelial Growth Factor A / therapeutic use

Substances

  • Anti-Bacterial Agents
  • Antibodies, Monoclonal, Humanized
  • Bacterial Toxins
  • Plasma Substitutes
  • Vascular Endothelial Growth Factor A
  • Shiga Toxin
  • Complement System Proteins
  • eculizumab