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A rare case of cutaneous involvement in atypical haemolytic uraemic syndrome successfully treated with eculizumab

Bibliographic Details
Main Author: Lopes,Daniela
Publication Date: 2016
Other Authors: Gomes,Ana Marta, Cunha,Cátia, Pereira,Susana, Tente,David, Fernandes,João Carlos
Format: Article
Language: eng
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000200010
Summary: Atypical haemolytic uraemic syndrome (aHUS) is a rare, life-threatening, genetic disease, due to uncontrolled alternative pathway complement activation. Although the renal microvasculature appears to be the predominantly affected target, other organ pathology compatible with local thrombotic microangiopathy has been reported. Eculizumab is a humanized antibody therapy that has been associated with significant inhibition of complement-mediated thrombotic microangiopathy events in aHUS. In this report, we describe the rare case of a patient with relapsing atypical haemolytic uraemic syndrome, cutaneous manifestations of the thrombotic microangiopathy and we discuss the treatment with plasma exchange and eculizumab
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spelling A rare case of cutaneous involvement in atypical haemolytic uraemic syndrome successfully treated with eculizumabAtypical haemolytic uraemic syndromecomplement mutationseculizumabAtypical haemolytic uraemic syndrome (aHUS) is a rare, life-threatening, genetic disease, due to uncontrolled alternative pathway complement activation. Although the renal microvasculature appears to be the predominantly affected target, other organ pathology compatible with local thrombotic microangiopathy has been reported. Eculizumab is a humanized antibody therapy that has been associated with significant inhibition of complement-mediated thrombotic microangiopathy events in aHUS. In this report, we describe the rare case of a patient with relapsing atypical haemolytic uraemic syndrome, cutaneous manifestations of the thrombotic microangiopathy and we discuss the treatment with plasma exchange and eculizumabSociedade Portuguesa de Nefrologia2016-06-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articletext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000200010Portuguese Journal of Nephrology & Hypertension v.30 n.2 2016reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000200010Lopes,DanielaGomes,Ana MartaCunha,CátiaPereira,SusanaTente,DavidFernandes,João Carlosinfo:eu-repo/semantics/openAccess2024-02-06T17:04:52Zoai:scielo:S0872-01692016000200010Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T12:54:28.253998Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv A rare case of cutaneous involvement in atypical haemolytic uraemic syndrome successfully treated with eculizumab
title A rare case of cutaneous involvement in atypical haemolytic uraemic syndrome successfully treated with eculizumab
spellingShingle A rare case of cutaneous involvement in atypical haemolytic uraemic syndrome successfully treated with eculizumab
Lopes,Daniela
Atypical haemolytic uraemic syndrome
complement mutations
eculizumab
title_short A rare case of cutaneous involvement in atypical haemolytic uraemic syndrome successfully treated with eculizumab
title_full A rare case of cutaneous involvement in atypical haemolytic uraemic syndrome successfully treated with eculizumab
title_fullStr A rare case of cutaneous involvement in atypical haemolytic uraemic syndrome successfully treated with eculizumab
title_full_unstemmed A rare case of cutaneous involvement in atypical haemolytic uraemic syndrome successfully treated with eculizumab
title_sort A rare case of cutaneous involvement in atypical haemolytic uraemic syndrome successfully treated with eculizumab
author Lopes,Daniela
author_facet Lopes,Daniela
Gomes,Ana Marta
Cunha,Cátia
Pereira,Susana
Tente,David
Fernandes,João Carlos
author_role author
author2 Gomes,Ana Marta
Cunha,Cátia
Pereira,Susana
Tente,David
Fernandes,João Carlos
author2_role author
author
author
author
author
dc.contributor.author.fl_str_mv Lopes,Daniela
Gomes,Ana Marta
Cunha,Cátia
Pereira,Susana
Tente,David
Fernandes,João Carlos
dc.subject.por.fl_str_mv Atypical haemolytic uraemic syndrome
complement mutations
eculizumab
topic Atypical haemolytic uraemic syndrome
complement mutations
eculizumab
description Atypical haemolytic uraemic syndrome (aHUS) is a rare, life-threatening, genetic disease, due to uncontrolled alternative pathway complement activation. Although the renal microvasculature appears to be the predominantly affected target, other organ pathology compatible with local thrombotic microangiopathy has been reported. Eculizumab is a humanized antibody therapy that has been associated with significant inhibition of complement-mediated thrombotic microangiopathy events in aHUS. In this report, we describe the rare case of a patient with relapsing atypical haemolytic uraemic syndrome, cutaneous manifestations of the thrombotic microangiopathy and we discuss the treatment with plasma exchange and eculizumab
publishDate 2016
dc.date.none.fl_str_mv 2016-06-01
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=S0872-01692016000200010
url http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000200010
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000200010
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.30 n.2 2016
reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
instacron:RCAAP
instname_str FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
instacron_str RCAAP
institution RCAAP
reponame_str Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
collection Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
repository.name.fl_str_mv Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
repository.mail.fl_str_mv info@rcaap.pt
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