title: Haemolytic uraemic syndrome.
abstract: Haemolytic uraemic syndrome is a form of thrombotic microangiopathy affecting predominantly the kidney and characterised by a triad of thrombocytopenia, mechanical haemolytic anaemia, and acute kidney injury. The term encompasses several disorders: shiga toxin-induced and pneumococcus-induced haemolytic uraemic syndrome, haemolytic uraemic syndrome associated with complement dysregulation or mutation of diacylglycerol kinase ɛ, haemolytic uraemic syndrome related to cobalamin C defect, and haemolytic uraemic syndrome secondary to a heterogeneous group of causes (infections, drugs, cancer, and systemic diseases). In the past two decades, experimental, genetic, and clinical studies have helped to decipher the pathophysiology of these various forms of haemolytic uraemic syndrome and undoubtedly improved diagnostic approaches. Moreover, a specific mechanism-based treatment has been made available for patients affected by atypical haemolytic uraemic syndrome due to complement dysregulation. Such treatment is, however, still absent for several other disease types, including shiga toxin-induced haemolytic uraemic syndrome.
authors.affiliation: Department of Nephrology, Centre Hospitalier Universitaire, and INSERM UMR S1064, Nantes, France.
authors.forename: Fadi
authors.initials: F
authors.lastname: Fakhouri
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Department of Nephrology and Renal Transplantation, Hôpital Necker, Université Paris Descartes, Paris, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Zuber
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Department of Biological Immunology, Hôpital Européen Georges Pompidou, and INSERM UMR S1138, Complément et Maladies, Centre de Recherche des Cordeliers, Paris, France.
authors.forename: Véronique
authors.initials: V
authors.lastname: Frémeaux-Bacchi
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Department of Pediatric Nephrology, Hôpital Robert Debré, Université Paris Diderot, Sorbonne Paris Cité, Paris, France. Electronic address: chantal.loirat@aphp.fr.
authors.forename: Chantal
authors.initials: C
authors.lastname: Loirat
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001688:Biological Products / Q000627:therapeutic use; D002648:Child; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D006801:Humans; D016030:Kidney Transplantation; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents; D001688:Biological Products