Atypical haemolytic-uraemic syndrome: reflecting over the old and new

Detalhes bibliográficos
Autor(a) principal: Farinha,Ana
Data de Publicação: 2012
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692012000300002
Resumo: Atypical haemolytic uraemic syndrome is a rare disease characterised by microangiopathic haemolytic anaemia, thrombocytopaenia and predominant renal impairment in the absence of Shiga toxin-producing bacteria. For long time it has been difficult to distinguish it from other thrombotic microangiopathies, but in the last decade advances have been made in understanding the pathogenesis of atypical haemolytic uraemic syndrome as a disorder of alternative pathway of the complement system. Knowledge of mutations and polymorphisms in the genes encoding the complement regulatory proteins revealed clinical importance in the management of the patients, altering not only the transplantation perspective but also leading to the search for new drugs, something that will potentially change the poor prognosis of these patients. This article reviews the differential diagnosis of this thrombotic microangiopathy to reflect on current treatment options and discuss new therapies
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spelling Atypical haemolytic-uraemic syndrome: reflecting over the old and newAlternative complement pathwayatypical haemolyticuraemic syndromeeculizumabplasmapheresistransplantationAtypical haemolytic uraemic syndrome is a rare disease characterised by microangiopathic haemolytic anaemia, thrombocytopaenia and predominant renal impairment in the absence of Shiga toxin-producing bacteria. For long time it has been difficult to distinguish it from other thrombotic microangiopathies, but in the last decade advances have been made in understanding the pathogenesis of atypical haemolytic uraemic syndrome as a disorder of alternative pathway of the complement system. Knowledge of mutations and polymorphisms in the genes encoding the complement regulatory proteins revealed clinical importance in the management of the patients, altering not only the transplantation perspective but also leading to the search for new drugs, something that will potentially change the poor prognosis of these patients. This article reviews the differential diagnosis of this thrombotic microangiopathy to reflect on current treatment options and discuss new therapiesSociedade Portuguesa de Nefrologia2012-07-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articletext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692012000300002Portuguese Journal of Nephrology & Hypertension v.26 n.3 2012reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692012000300002Farinha,Anainfo:eu-repo/semantics/openAccess2024-02-06T17:04:39Zoai:scielo:S0872-01692012000300002Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:18:49.153663Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Atypical haemolytic-uraemic syndrome: reflecting over the old and new
title Atypical haemolytic-uraemic syndrome: reflecting over the old and new
spellingShingle Atypical haemolytic-uraemic syndrome: reflecting over the old and new
Farinha,Ana
Alternative complement pathway
atypical haemolyticuraemic syndrome
eculizumab
plasmapheresis
transplantation
title_short Atypical haemolytic-uraemic syndrome: reflecting over the old and new
title_full Atypical haemolytic-uraemic syndrome: reflecting over the old and new
title_fullStr Atypical haemolytic-uraemic syndrome: reflecting over the old and new
title_full_unstemmed Atypical haemolytic-uraemic syndrome: reflecting over the old and new
title_sort Atypical haemolytic-uraemic syndrome: reflecting over the old and new
author Farinha,Ana
author_facet Farinha,Ana
author_role author
dc.contributor.author.fl_str_mv Farinha,Ana
dc.subject.por.fl_str_mv Alternative complement pathway
atypical haemolyticuraemic syndrome
eculizumab
plasmapheresis
transplantation
topic Alternative complement pathway
atypical haemolyticuraemic syndrome
eculizumab
plasmapheresis
transplantation
description Atypical haemolytic uraemic syndrome is a rare disease characterised by microangiopathic haemolytic anaemia, thrombocytopaenia and predominant renal impairment in the absence of Shiga toxin-producing bacteria. For long time it has been difficult to distinguish it from other thrombotic microangiopathies, but in the last decade advances have been made in understanding the pathogenesis of atypical haemolytic uraemic syndrome as a disorder of alternative pathway of the complement system. Knowledge of mutations and polymorphisms in the genes encoding the complement regulatory proteins revealed clinical importance in the management of the patients, altering not only the transplantation perspective but also leading to the search for new drugs, something that will potentially change the poor prognosis of these patients. This article reviews the differential diagnosis of this thrombotic microangiopathy to reflect on current treatment options and discuss new therapies
publishDate 2012
dc.date.none.fl_str_mv 2012-07-01
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692012000300002
url http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692012000300002
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692012000300002
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv text/html
dc.publisher.none.fl_str_mv Sociedade Portuguesa de Nefrologia
publisher.none.fl_str_mv Sociedade Portuguesa de Nefrologia
dc.source.none.fl_str_mv Portuguese Journal of Nephrology & Hypertension v.26 n.3 2012
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
repository.mail.fl_str_mv
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