Update on Coronary Involvement in Fabry Disease

Detalhes bibliográficos
Autor(a) principal: Cabrera,Gustavo
Data de Publicação: 2016
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Journal of Inborn Errors of Metabolism and Screening
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942016000100801
Resumo: Abstract Fabry disease is a multisystemic disorder with consequent morbidity and mortality at an early age in patients of both genders. Although renal failure has been previously described as the cause of death with the highest prevalence, recent studies, based on the analysis of the population recorded in the Fabry Registry, reported that 40% of deaths had a cardiovascular origin. Data from the same registry emphasize the high risk for these patients suffering cardiovascular events—particularly acute myocardial infarction—and to the fact that this risk is higher for those patients who need dialysis. Microvascular dysfunction is a constant in patients with Fabry disease, which affects young patients, independently from other cardiac involvement manifestations—such as left ventricular hypertrophy—and from the patient’s gender.
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spelling Update on Coronary Involvement in Fabry DiseaseFabry diseasecoronary involvementmicrovascular involvementcoronary syndromesacute myocardial infarctioncoronary revascularizationAbstract Fabry disease is a multisystemic disorder with consequent morbidity and mortality at an early age in patients of both genders. Although renal failure has been previously described as the cause of death with the highest prevalence, recent studies, based on the analysis of the population recorded in the Fabry Registry, reported that 40% of deaths had a cardiovascular origin. Data from the same registry emphasize the high risk for these patients suffering cardiovascular events—particularly acute myocardial infarction—and to the fact that this risk is higher for those patients who need dialysis. Microvascular dysfunction is a constant in patients with Fabry disease, which affects young patients, independently from other cardiac involvement manifestations—such as left ventricular hypertrophy—and from the patient’s gender.Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT)2016-01-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942016000100801Journal of Inborn Errors of Metabolism and Screening v.4 2016reponame:Journal of Inborn Errors of Metabolism and Screeninginstname:Instituto Genética para Todos (IGPT)instacron:IGPT10.1177/2326409816679427info:eu-repo/semantics/openAccessCabrera,Gustavoeng2019-05-28T00:00:00Zoai:scielo:S2326-45942016000100801Revistahttp://jiems-journal.org/ONGhttps://old.scielo.br/oai/scielo-oai.phpjiems@jiems-journal.org||rgiugliani@hcpa.edu.br2326-45942326-4594opendoar:2019-05-28T00:00Journal of Inborn Errors of Metabolism and Screening - Instituto Genética para Todos (IGPT)false
dc.title.none.fl_str_mv Update on Coronary Involvement in Fabry Disease
title Update on Coronary Involvement in Fabry Disease
spellingShingle Update on Coronary Involvement in Fabry Disease
Cabrera,Gustavo
Fabry disease
coronary involvement
microvascular involvement
coronary syndromes
acute myocardial infarction
coronary revascularization
title_short Update on Coronary Involvement in Fabry Disease
title_full Update on Coronary Involvement in Fabry Disease
title_fullStr Update on Coronary Involvement in Fabry Disease
title_full_unstemmed Update on Coronary Involvement in Fabry Disease
title_sort Update on Coronary Involvement in Fabry Disease
author Cabrera,Gustavo
author_facet Cabrera,Gustavo
author_role author
dc.contributor.author.fl_str_mv Cabrera,Gustavo
dc.subject.por.fl_str_mv Fabry disease
coronary involvement
microvascular involvement
coronary syndromes
acute myocardial infarction
coronary revascularization
topic Fabry disease
coronary involvement
microvascular involvement
coronary syndromes
acute myocardial infarction
coronary revascularization
description Abstract Fabry disease is a multisystemic disorder with consequent morbidity and mortality at an early age in patients of both genders. Although renal failure has been previously described as the cause of death with the highest prevalence, recent studies, based on the analysis of the population recorded in the Fabry Registry, reported that 40% of deaths had a cardiovascular origin. Data from the same registry emphasize the high risk for these patients suffering cardiovascular events—particularly acute myocardial infarction—and to the fact that this risk is higher for those patients who need dialysis. Microvascular dysfunction is a constant in patients with Fabry disease, which affects young patients, independently from other cardiac involvement manifestations—such as left ventricular hypertrophy—and from the patient’s gender.
publishDate 2016
dc.date.none.fl_str_mv 2016-01-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942016000100801
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942016000100801
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1177/2326409816679427
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 Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT)
publisher.none.fl_str_mv Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT)
dc.source.none.fl_str_mv Journal of Inborn Errors of Metabolism and Screening v.4 2016
reponame:Journal of Inborn Errors of Metabolism and Screening
instname:Instituto Genética para Todos (IGPT)
instacron:IGPT
instname_str Instituto Genética para Todos (IGPT)
instacron_str IGPT
institution IGPT
reponame_str Journal of Inborn Errors of Metabolism and Screening
collection Journal of Inborn Errors of Metabolism and Screening
repository.name.fl_str_mv Journal of Inborn Errors of Metabolism and Screening - Instituto Genética para Todos (IGPT)
repository.mail.fl_str_mv jiems@jiems-journal.org||rgiugliani@hcpa.edu.br
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