The Haemolytic Uraemic Syndrome

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Recent advances in understanding the aetiology of the disorders that make up the haemolytic uraemic syndrome (HUS) permit a revised classification of the syndrome. With appropriate laboratory support, an aetiologically-based subgroup diagnosis can be made in all but a few cases. HUS caused by entero...

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Autor principal: Cuestas, Eduardo
Formato: Artículo revista
Lenguaje:Español
Publicado: Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2020
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Acceso en línea:https://revistas.unc.edu.ar/index.php/med/article/view/27716
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Revistas de la UNC de Universidad Nacional de Córdoba
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spelling I10-R10-article-277162020-03-05T18:50:00Z The Haemolytic Uraemic Syndrome Síndrome Urémico Hemólitico Cuestas, Eduardo . Recent advances in understanding the aetiology of the disorders that make up the haemolytic uraemic syndrome (HUS) permit a revised classification of the syndrome. With appropriate laboratory support, an aetiologically-based subgroup diagnosis can be made in all but a few cases. HUS caused by enterohaemorrhagic Escherichia coli remains by far the most prevalent subgroup, and new insights into this zoonosis are discussed. The most rapidly expanding area of interest is the subgroup of inherited and acquired abnormalities of complement regulation. Details of the pathogenesis are incomplete but it is reasonable to conclude that local activation of the alternative pathway of complement in the glomerulus is a central event. There is no evidence-based treatment for this diagnostic subgroup. However, in circumstances where there is a mutated plasma factor such as complement factor H, strategies to replace the abnormal protein by plasmapheresis or more radically by liver transplantation are logical, and anecdotal successes are reported. In summary, the clinical presentation of HUS gives a strong indication as to the underlying cause. Patients without evidence of EHEC infection should be fully investigated to determine the aetiology. Where complement abnormalities are suspected there is a strong argument for empirical and early plasma exchange, although rapid advances in this field may provide more specific treatments in the near future. Los recientes avances en la comprensión de la etiología de los trastornos que componen elsíndrome urémico hemolítico (síndrome urémico hemolítico) permiten una clasificación revisada delsíndrome. Con apoyo de un laboratorio apropiado, un diagnóstico etiológico puede ser hecho encasi todos los casos. El síndrome urémico hemolítico causado por Escherichia colienterohemorrágica (ECEH) sigue siendo, con mucho, el subgrupo más frecuente, y se discuten losnuevos conocimientos sobre esta zoonosis. El subgrupo de más rápida expansión de área delinterés es el subgrupo de las anomalías heredadas y adquiridas del complemento. Aunque algunosdetalles de la patogénesis son aún incompletos, es razonable concluir que la activación de la víaalterna del complemento en el glomérulo es un evento central. No hay pruebas suficientes parafundamentar el tratamiento de este subgrupo de diagnóstico. Sin embargo, en circunstancias en lasque existe un factor mutado como el factor de complemento H, las estrategias para sustituir laproteína anormal es la plasmaféresis o más radicalmente el trasplante hepático pudiera parecerlógico, se han informado éxitos anecdóticos. En resumen, la presentación clínica de síndromeurémico hemolítico puede dar indicios claros de la causa subyacente. Los pacientes sin evidenciade infección por ECEH deben ser plenamente investigados para determinar la etiología. Cuando sesospeche la existencia de anomalías del complemento existe un fuerte argumento empírico enfavor de la plasmaféresis. Los rápidos avances en este campo seguramente proporcionarántratamientos más específicos en un futuro próximo Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2020-02-18 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion application/pdf https://revistas.unc.edu.ar/index.php/med/article/view/27716 Revista de la Facultad de Ciencias Médicas de Córdoba.; Vol. 65 No. 2 (2008); 54 - 58 Revista de la Facultad de Ciencias Médicas de Córdoba; Vol. 65 Núm. 2 (2008); 54 - 58 Revista da Faculdade de Ciências Médicas de Córdoba; v. 65 n. 2 (2008); 54 - 58 1853-0605 0014-6722 spa https://revistas.unc.edu.ar/index.php/med/article/view/27716/29127 Derechos de autor 2020 Universidad Nacional de Córdoba
institution Universidad Nacional de Córdoba
institution_str I-10
repository_str R-10
container_title_str Revistas de la UNC
language Español
format Artículo revista
topic .
spellingShingle .
Cuestas, Eduardo
The Haemolytic Uraemic Syndrome
topic_facet .
author Cuestas, Eduardo
author_facet Cuestas, Eduardo
author_sort Cuestas, Eduardo
title The Haemolytic Uraemic Syndrome
title_short The Haemolytic Uraemic Syndrome
title_full The Haemolytic Uraemic Syndrome
title_fullStr The Haemolytic Uraemic Syndrome
title_full_unstemmed The Haemolytic Uraemic Syndrome
title_sort haemolytic uraemic syndrome
description Recent advances in understanding the aetiology of the disorders that make up the haemolytic uraemic syndrome (HUS) permit a revised classification of the syndrome. With appropriate laboratory support, an aetiologically-based subgroup diagnosis can be made in all but a few cases. HUS caused by enterohaemorrhagic Escherichia coli remains by far the most prevalent subgroup, and new insights into this zoonosis are discussed. The most rapidly expanding area of interest is the subgroup of inherited and acquired abnormalities of complement regulation. Details of the pathogenesis are incomplete but it is reasonable to conclude that local activation of the alternative pathway of complement in the glomerulus is a central event. There is no evidence-based treatment for this diagnostic subgroup. However, in circumstances where there is a mutated plasma factor such as complement factor H, strategies to replace the abnormal protein by plasmapheresis or more radically by liver transplantation are logical, and anecdotal successes are reported. In summary, the clinical presentation of HUS gives a strong indication as to the underlying cause. Patients without evidence of EHEC infection should be fully investigated to determine the aetiology. Where complement abnormalities are suspected there is a strong argument for empirical and early plasma exchange, although rapid advances in this field may provide more specific treatments in the near future.
publisher Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
publishDate 2020
url https://revistas.unc.edu.ar/index.php/med/article/view/27716
work_keys_str_mv AT cuestaseduardo thehaemolyticuraemicsyndrome
AT cuestaseduardo sindromeuremicohemolitico
AT cuestaseduardo haemolyticuraemicsyndrome
first_indexed 2022-08-20T01:27:17Z
last_indexed 2022-08-20T01:27:17Z
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