title: Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome.
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.
authors.affiliation: Pharmacie usage intérieur, hôpital Timone, AP-HM, 13005 Marseille, France.
authors.forename: C
authors.initials: C
authors.lastname: Picard
authors.affiliation: Centre de néphrologie et de transplantation rénale, hôpital de la Conception, AP-HM, 13005 Marseille, France.
authors.forename: S
authors.initials: S
authors.lastname: Burtey
authors.affiliation: Pharmacie usage intérieur, hôpital de la Conception, AP-HM, 13005 Marseille, France.
authors.forename: C
authors.initials: C
authors.lastname: Bornet
authors.affiliation: Service central de la qualité et de l'information pharmaceutiques (SCQIP), AP-HM, 13005 Marseille, France; Institut de chimie radicalaire ICR, UMR 7273, laboratoire de pharmaco-chimie radicalaire, CNRS, Aix-Marseille université, 13385 Marseille, France.
authors.forename: C
authors.initials: C
authors.lastname: Curti
authors.affiliation: Oncopharma, AP-HM, 13005 Marseille, France; Institut de chimie radicalaire ICR, UMR 7273, laboratoire de pharmaco-chimie radicalaire, CNRS, Aix-Marseille université, 13385 Marseille, France.
authors.forename: M
authors.initials: M
authors.lastname: Montana
authors.affiliation: Service central de la qualité et de l'information pharmaceutiques (SCQIP), AP-HM, 13005 Marseille, France; Institut de chimie radicalaire ICR, UMR 7273, laboratoire de pharmaco-chimie radicalaire, CNRS, Aix-Marseille université, 13385 Marseille, France. Electronic address: patrice.vanelle@univ-amu.fr.
authors.forename: P
authors.initials: P
authors.lastname: Vanelle
mesh_terms: D000818:Animals; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D001424:Bacterial Infections / Q000150:complications; D001427:Bacterial Toxins / Q000009:adverse effects; D002986:Clinical Trials as Topic; D003165:Complement System Proteins / Q000502:physiology; D004195:Disease Models, Animal; D004353:Drug Evaluation, Preclinical; D004927:Escherichia coli Infections / Q000150:complications / Q000382:microbiology; D005544:Forecasting; D020022:Genetic Predisposition to Disease; D006463:Hemolytic-Uremic Syndrome / Q000145:classification / Q000209:etiology / Q000235:genetics / Q000382:microbiology / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D016030:Kidney Transplantation; D016031:Liver Transplantation; D051379:Mice; D010215:Papio; D010949:Plasma; D010952:Plasma Substitutes; D022621:Shiga Toxin / Q000009:adverse effects; D054323:Shiga-Toxigenic Escherichia coli / Q000276:immunology / Q000472:pathogenicity; D019851:Thrombophilia / Q000209:etiology; D042461:Vascular Endothelial Growth Factor A / Q000627:therapeutic use
chemical_list: D000900:Anti-Bacterial Agents; D061067:Antibodies, Monoclonal, Humanized; D001427:Bacterial Toxins; D010952:Plasma Substitutes; D042461:Vascular Endothelial Growth Factor A; D022621:Shiga Toxin; D003165:Complement System Proteins; C481642:eculizumab
keywords: Analogue des récepteurs aux Shiga toxines; Eculizumab; Hemolytic uremic syndrome; Shiga toxin receptor analogues; Syndrome hémolytique urémique; Urtoxazumab