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Title: An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document
Other Titles: Actualización en síndrome hemolítico urémico atípico: diagnóstico y tratamiento. Documento de consenso
Author: Campistol Plana, Josep M.
Arias, Manuel
Ariceta, Gema
Blasco, Miguel
Espinosa, Mario
Grinyo Boira, Josep M.
Praga, Manuel
Torra, Roser
Vilalta, Ramon (Vilalta Casas)
Rodríguez de Córdoba, Santiago
Keywords: Anèmia hemolítica
Trastorns de les plaquetes sanguínies
Insuficiència renal aguda
Malalties hematològiques
Anticossos monoclonals
Hemolytic anemia
Blood platelet disorders
Acute renal failure
Hematologic diseases
Monoclonal antibodies
Issue Date: 2013
Publisher: Elsevier España
Abstract: Haemolytic uraemic syndrome (HUS) is a clinical entity defined as the triad of nonimmune haemolytic anaemia, thrombocytopenia, and acute renal failure, in which the underlying lesions are mediated by systemic thrombotic microangiopathy (TMA). Atypical HUS (aHUS) is a sub-type of HUS in which the TMA phenomena are the consequence of decreased regulation of the alternative complement pathway on cell surfaces due to a genetic cause. aHUS is an extremely rare disease that, despite the administration of standard treatment with plasma therapy, often progresses to terminal chronic renal failure with a high associated rate of mortality. In recent years, research has established the key role that the complement system plays in the induction of endothelial damage in patients with aHUS, through the characterisation of multiple mutations and polymorphisms in the genes that code for certain complement factors. Eculizumab is a monoclonal antibody that inhibits the terminal fraction of the complement protein, blocking the formation of a cell membrane attack complex. In prospective studies in patients with aHUS, administering eculizumab produces a rapid and sustained interruption in the TMA process, with significant improvements in long-term renal function and an important decrease in the need for dialysis or plasma therapy. In this document, we review and bring up to date the important aspects of this disease, with special emphasis on how recent advancements in diagnostic and therapeutic processes can modify the treatment of patients with aHUS.
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It is part of: Nefrología, 2013, vol. 33, num. 1, p. 27-45
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ISSN: 0211-6995
Appears in Collections:Articles publicats en revistes (Ciències Clíniques)
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Medicina)

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