title: Haemolytic uraemic syndrome.
abstract: Haemolytic uraemic syndrome (HUS) is defined by the simultaneous occurrence of nonimmune haemolytic anaemia, thrombocytopenia and acute renal failure. This leads to the pathological lesion termed thrombotic microangiopathy, which mainly affects the kidney, as well as other organs. HUS is associated with endothelial cell injury and platelet activation, although the underlying cause may differ. Most cases of HUS are associated with gastrointestinal infection with Shiga toxin-producing enterohaemorrhagic Escherichia coli (EHEC) strains. Atypical HUS (aHUS) is associated with complement dysregulation due to mutations or autoantibodies. In this review, we will describe the causes of HUS. In addition, we will review the clinical, pathological, haematological and biochemical features, epidemiology and pathogenetic mechanisms as well as the biochemical, microbiological, immunological and genetic investigations leading to diagnosis. Understanding the underlying mechanisms of the different subtypes of HUS enables tailoring of appropriate treatment and management. To date, there is no specific treatment for EHEC-associated HUS but patients benefit from supportive care, whereas patients with aHUS are effectively treated with anti-C5 antibody to prevent recurrences, both before and after renal transplantation.
authors.affiliation: Department of Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden.
authors.forename: Diana
authors.identifier: 0000-0001-7220-7658
authors.initials: D
authors.lastname: Karpman
authors.affiliation: Department of Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Loos
authors.affiliation: Department of Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden.
authors.forename: Ramesh
authors.initials: R
authors.lastname: Tati
authors.affiliation: Department of Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden.
authors.forename: Ida
authors.initials: I
authors.lastname: Arvidsson
mesh_terms: D003937:Diagnosis, Differential; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D011379:Prognosis
keywords: Shiga toxin; complement; enterohaemorrhagic Escherichia coli; haemolytic uraemic syndrome; microvesicles