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Atypical Hemolytic-Uremic Syndrome: An Update on Pathophysiology, Diagnosis, and Treatment.
Raina, Rupesh; Krishnappa, Vinod; Blaha, Taryn; Kann, Taylor; Hein, William; Burke, Linda; Bagga, Arvind.
Afiliação
  • Raina R; Department of Nephrology, Cleveland Clinic Akron General and Akron Children's Hospital, Akron, OH, USA.
  • Krishnappa V; Cleveland Clinic Akron General/Akron Nephrology Associates, Akron, OH, USA.
  • Blaha T; Cleveland Clinic Akron General/Akron Nephrology Associates, Akron, OH, USA.
  • Kann T; Northeast Ohio Medical University, Rootstown, OH, USA.
  • Hein W; Department of Internal Medicine, Cleveland Clinic Akron General, Akron, OH, USA.
  • Burke L; Department of Internal Medicine, Cleveland Clinic Akron General, Akron, OH, USA.
  • Bagga A; Ohio University Heritage College of Osteopathic Medicine, Athens, OH, USA.
Ther Apher Dial ; 23(1): 4-21, 2019 Feb.
Article em En | MEDLINE | ID: mdl-30294946
Atypical hemolytic uremic syndrome (aHUS), a rare variant of thrombotic microangiopathy, is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. The condition is associated with poor clinical outcomes with high morbidity and mortality. Atypical HUS predominantly affects the kidneys but has the potential to cause multi-organ system dysfunction. This uncommon disorder is caused by a genetic abnormality in the complement alternative pathway resulting in over-activation of the complement system and formation of microvascular thrombi. Abnormalities of the complement pathway may be in the form of mutations in key complement genes or autoantibodies against specific complement factors. We discuss the pathophysiology, clinical manifestations, diagnosis, complications, and management of aHUS. We also review the efficacy and safety of the novel therapeutic agent, eculizumab, in aHUS, pregnancy-associated aHUS, and aHUS in renal transplant patients.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Via Alternativa do Complemento / Anticorpos Monoclonais Humanizados / Síndrome Hemolítico-Urêmica Atípica Tipo de estudo: Diagnostic_studies / Etiology_studies Limite: Humans Idioma: En Revista: Ther Apher Dial Assunto da revista: HEMATOLOGIA Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Estados Unidos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Via Alternativa do Complemento / Anticorpos Monoclonais Humanizados / Síndrome Hemolítico-Urêmica Atípica Tipo de estudo: Diagnostic_studies / Etiology_studies Limite: Humans Idioma: En Revista: Ther Apher Dial Assunto da revista: HEMATOLOGIA Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Estados Unidos