Consenso latinoamericano de síndrome hemolítico urémico atípico

datacite.rightshttp://purl.org/coar/access_right/c_abf2
dc.contributor.authorNieto-Ríos, John F.
dc.contributor.authorBello Márquez, Diana Carolina
dc.contributor.authorMORALES-BUENROSTRO, LUIS
dc.contributor.authorMusalem, Pilar
dc.contributor.authorVaisbich, Maria Helena
dc.contributor.authorFortunato, Rita M.
dc.contributor.authorGómez-Navarro, Benjamín
dc.contributor.authorJiménez-Acosta, Darío
dc.contributor.authorMaldonado , Rafael Alberto
dc.contributor.authorTrimarchi, Hernán
dc.contributor.authorRico-Fontalvo, Jorge
dc.date.accessioned2025-10-30T15:27:51Z
dc.date.available2025-10-30T15:27:51Z
dc.date.issued2025
dc.description.abstractEl síndrome hemolítico urémico atípico primario es una microangiopatía trombótica relacionada con una alteración en la regulación de la vía alterna del complemento, caracterizada por daño endotelial difuso, hemólisis microangiopática y compromiso orgánico multisistémico, ocasionando una alta morbimortalidad. Su diagnóstico diferencial incluye la púrpura trombocitopénica trombótica, el síndrome hemolítico urémico relacionado con la toxina Shiga y las microangiopatías trombóticas secundarias. En su evaluación son fundamentales la medición de la actividad de la enzima ADAMTS-13, la determinación de la toxina Shiga en materia fecal o con pruebas moleculares que identifiquen las bacterias productoras de esta toxina, el estudio de enfermedades sistémicas que causan microangiopatías trombóticas y la realización de pruebas genéticas de la regulación de la vía alterna del complemento. El tratamiento de elección es la inhibición de la fracción C5 del complemento con fármacos como el eculizumab y el ravulizumab. El pronóstico depende de un diagnóstico temprano y de un tratamiento oportuno. En Latinoamérica, este síndrome ha sido escasamente estudiado y hay pocos consensos que unifiquen los criterios de evaluación y manejo.spa
dc.description.abstractPrimary atypical hemolytic uremic syndrome is a thrombotic microangiopathy associated with altered regulation of the alternate complement pathway. It is characterized by diffuse endothelial damage, microangiopathic hemolysis, and multisystem organ involvement, causing high morbidity and mortality. Its differential diagnosis includes thrombotic thrombocytopenic purpura, Shiga toxin-associated hemolytic uremic syndrome, and secondary thrombotic microangiopathies. Its evaluation includes measuring ADAMTS-13 enzyme activity, determining Shiga toxin in fecal matter, or using molecular tests to identify the bacteria that produce this toxin. Its evaluation also includes the study of systemic diseases that cause thrombotic microangiopathies and performing genetic testing for regulation of the alternate complement pathway. The treatment of choice is inhibition of the C5 fraction of complement with drugs such as eculizumab and ravulizumab. The prognosis depends on early diagnosis and timely treatment. In Latin America, this syndrome has been poorly studied, and there is little consensus that unifies evaluation and management criteria.eng
dc.format.mimetypepdf
dc.identifier.doi10.24875/NEFRO.M25000070
dc.identifier.issn24449032
dc.identifier.urihttps://hdl.handle.net/20.500.12442/17066
dc.identifier.urlhttps://www.nefrologialatinoamericana.com/portadas/nefro_25_22_supl-2.pdf
dc.language.isospa
dc.publisherSociedad Latinoamericana de Nefrología e Hipertensiónspa
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internationaleng
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.sourceNefrología Latinoamericanaspa
dc.subjectMicroangiopatías trombóticasspa
dc.subjectSíndrome hemolítico urémico atípicospa
dc.subjectIntercambio plasmáticospa
dc.subjectEculizumabspa
dc.subjectRavulizumabspa
dc.subject.keywordsThrombotic microangiopathieseng
dc.subject.keywordsAtypical Hemolytic Uremic Syndromeeng
dc.subject.keywordsPlasma exchangeeng
dc.titleConsenso latinoamericano de síndrome hemolítico urémico atípicospa
dc.title.translatedLatin American consensus on atypical hemolytic uremic syndromeeng
dc.type.driverinfo:eu-repo/semantics/article
dc.type.spaArtículo científico
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