Atypical Hemolytic Uremic Syndrome, literature revision and consensus document. Diagnosis and treatment
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Keywords

Atypical Hemolytic Uremic Syndrome
Complement
Thrombotic microangiopathy
Plasmapheresis
Eculizumab
Renal transplant

How to Cite

1.
Córdoba JP, Contreras KM, Larrarte C, Espitaleta Z, González LE, Ibarra M, Echeverri JE, Carrascal M, Valderrama LA, Prada M. Atypical Hemolytic Uremic Syndrome, literature revision and consensus document. Diagnosis and treatment. Rev. Colomb. Nefrol. [Internet]. 2015 Mar. 6 [cited 2022 May 18];2(1):19-40. Available from: https://revistanefrologia.org/index.php/rcn/article/view/199

Abstract

Atypical Hemolytic Uremic Syndrome is an ultra-orphan disease, more than 50% of patients die, need renal replacement therapy or have permanent renal failure within the first year of diagnostic. With current supportive care 9-15% of aHUS patients die within 1 year following a clinical manifestation of aHUS. Severe consequences of this disease reinforce the early diagnostic and treatment importance. Clinical manifestations of this disease include the classic triad of microangiopatic anemia, thrombocytopenia and end organ damage where the renal failure is the most common manifestation, although not the only one as neurological, cardiac and gastrointestinal complications are also apparent. Mutations on the complement system regulating proteins are recognized as the cause of this syndrome; however they are not identified in all patients as new mutations are continuously being identified. It has a high rate post-transplantation graft loss in 60% of the cases. Most known therapy for this disease, considered as the first line therapy was plasmapheresis; however it shows very poor results. Since 2011 we have a recombinant monoclonal antibody targeted to the complement component C5 (eculizumab), the only approved for the treatment of aHUS, which has proven to significantly improve the disease prognosis and progression, and is considered the first line therapy.
https://doi.org/10.22265/acnef.2.1.199
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