title: Posttransplant recurrence of atypical hemolytic uremic syndrome.
abstract: Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It is usually secondary to infections by strains of Escherichia coli (STEC) that produce Shiga-like toxin. In about 10% of patients, no STEC infections are reported. In these cases of atypical HUS (aHUS), mutations in genes encoding proteins of the complement system have been described. Atypical HUS is characterized by poor prognosis and by high risk of posttransplant recurrence which greatly depends on the specific gene mutation involved in the disease. Plasma therapy, eculizumab treatment and, in some cases, combined liver-kidney transplant have been used to prevent and/or treat posttransplant aHUS recurrences.
authors: Clinical Research Center for Rare Diseases Aldo e Cele Daccò, Mario Negri Institute for Pharmacological Research, Ranica, Bergamo, Italy.
authors: Elisabetta
authors: E
authors: Valoti
authors: Marta
authors: M
authors: Alberti
authors: Marina
authors: M
authors: Noris
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology / Q000276:immunology / Q000601:surgery*; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D065766:Atypical Hemolytic Uremic Syndrome; D003167:Complement Activation / Q000235:genetics; D003165:Complement System Proteins / Q000235:genetics; D020022:Genetic Predisposition to Disease; D006463:Hemolytic-Uremic Syndrome / Q000150:complications* / Q000235:genetics / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation / Q000009:adverse effects*; D016031:Liver Transplantation; D009154:Mutation; D010641:Phenotype; D010951:Plasma Exchange; D055502:Secondary Prevention; D016896:Treatment Outcome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D007166:Immunosuppressive Agents; D003165:Complement System Proteins; C481642:eculizumab