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Claves diagnósticas en el síndrome hemolítico urémico atípico: a propósito de un caso

Atypical hemolytic uremic syndrome (aHUS) is a clinical entity characterized by non-immune hemolytic anemia, thrombocytopenia and renal failure, in which lesions are mediated by a systemic thrombotic microangiopathy. It is a rare pathology whose origin is a complement system deregulation due to muta...

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Autores principales: Luquin Irigoyen, M., Armendariz Brugos, C., Vallejo Ruiz, M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Gobierno de Navarra. Departamento de Salud 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10130794/
https://www.ncbi.nlm.nih.gov/pubmed/35975546
http://dx.doi.org/10.23938/ASSN.1006
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author Luquin Irigoyen, M.
Armendariz Brugos, C.
Vallejo Ruiz, M.
author_facet Luquin Irigoyen, M.
Armendariz Brugos, C.
Vallejo Ruiz, M.
author_sort Luquin Irigoyen, M.
collection PubMed
description Atypical hemolytic uremic syndrome (aHUS) is a clinical entity characterized by non-immune hemolytic anemia, thrombocytopenia and renal failure, in which lesions are mediated by a systemic thrombotic microangiopathy. It is a rare pathology whose origin is a complement system deregulation due to mutations in its genes that lead to uncontrolled activation of C5 and the formation of the membrane attack complex.- Its correct diagnosis allows us to prescribe the treatment based on Eculizumab, a C5 inhibitor. We report the case of a pregnant patient with aHUS, with the aim of highlighting the importance of early differential diagnosis to establish an early and effective treatment of this pathology. The pathophysiology, diagnosis and genetic study are updated, as well as the therapeutic management of aHUS.
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spelling pubmed-101307942023-04-27 Claves diagnósticas en el síndrome hemolítico urémico atípico: a propósito de un caso Luquin Irigoyen, M. Armendariz Brugos, C. Vallejo Ruiz, M. An Sist Sanit Navar Notas Clínicas Atypical hemolytic uremic syndrome (aHUS) is a clinical entity characterized by non-immune hemolytic anemia, thrombocytopenia and renal failure, in which lesions are mediated by a systemic thrombotic microangiopathy. It is a rare pathology whose origin is a complement system deregulation due to mutations in its genes that lead to uncontrolled activation of C5 and the formation of the membrane attack complex.- Its correct diagnosis allows us to prescribe the treatment based on Eculizumab, a C5 inhibitor. We report the case of a pregnant patient with aHUS, with the aim of highlighting the importance of early differential diagnosis to establish an early and effective treatment of this pathology. The pathophysiology, diagnosis and genetic study are updated, as well as the therapeutic management of aHUS. Gobierno de Navarra. Departamento de Salud 2022-08-17 /pmc/articles/PMC10130794/ /pubmed/35975546 http://dx.doi.org/10.23938/ASSN.1006 Text en https://creativecommons.org/licenses/by-nc/3.0/Este es un artículo publicado en acceso abierto bajo una licencia Creative Commons
spellingShingle Notas Clínicas
Luquin Irigoyen, M.
Armendariz Brugos, C.
Vallejo Ruiz, M.
Claves diagnósticas en el síndrome hemolítico urémico atípico: a propósito de un caso
title Claves diagnósticas en el síndrome hemolítico urémico atípico: a propósito de un caso
title_full Claves diagnósticas en el síndrome hemolítico urémico atípico: a propósito de un caso
title_fullStr Claves diagnósticas en el síndrome hemolítico urémico atípico: a propósito de un caso
title_full_unstemmed Claves diagnósticas en el síndrome hemolítico urémico atípico: a propósito de un caso
title_short Claves diagnósticas en el síndrome hemolítico urémico atípico: a propósito de un caso
title_sort claves diagnósticas en el síndrome hemolítico urémico atípico: a propósito de un caso
topic Notas Clínicas
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10130794/
https://www.ncbi.nlm.nih.gov/pubmed/35975546
http://dx.doi.org/10.23938/ASSN.1006
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