Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients
Autor(a) principal: | |
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Data de Publicação: | 2015 |
Outros Autores: | , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Arquivos Brasileiros de Cardiologia (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2015002400503 |
Resumo: | Background:Familial amyloidotic polyneuropathy (FAP) is a rare disease diagnosed in Brazil and worldwide. The frequency of cardiovascular involvement in Brazilian FAP patients is unknown.Objective:Detect the frequency of cardiovascular involvement and correlate the cardiovascular findings with the modified polyneuropathy disability (PND) score.Methods:In a national reference center, 51 patients were evaluated with clinical examination, electrocardiography (ECG), echocardiography (ECHO), and 24-hour Holter. Patients were classified according to the modified PND score and divided into groups: PND 0, PND I, PND II, and PND > II (which included PND IIIa, IIIb, and IV). We chose the classification tree as the statistical method to analyze the association between findings in cardiac tests with the neurological classification (PND).Results:ECG abnormalities were present in almost 2/3 of the FAP patients, whereas ECHO abnormalities occurred in around 1/3 of them. All patients with abnormal ECHO also had abnormal ECG, but the opposite did not apply. The classification tree identified ECG and ECHO as relevant variables (p < 0.001 and p = 0.08, respectively). The probability of a patient to be allocated to the PND 0 group when having a normal ECG was over 80%. When both ECG and ECHO were abnormal, this probability was null.Conclusions:Brazilian patients with FAP have frequent ECG abnormalities. ECG is an appropriate test to discriminate asymptomatic carriers of the mutation from those who develop the disease, whereas ECHO contributes to this discrimination. |
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Arquivos Brasileiros de Cardiologia (Online) |
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Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian PatientsAmyloid NeuropathiesFamilialCardiomyopathiesPrealbuminEletrocardiographyBackground:Familial amyloidotic polyneuropathy (FAP) is a rare disease diagnosed in Brazil and worldwide. The frequency of cardiovascular involvement in Brazilian FAP patients is unknown.Objective:Detect the frequency of cardiovascular involvement and correlate the cardiovascular findings with the modified polyneuropathy disability (PND) score.Methods:In a national reference center, 51 patients were evaluated with clinical examination, electrocardiography (ECG), echocardiography (ECHO), and 24-hour Holter. Patients were classified according to the modified PND score and divided into groups: PND 0, PND I, PND II, and PND > II (which included PND IIIa, IIIb, and IV). We chose the classification tree as the statistical method to analyze the association between findings in cardiac tests with the neurological classification (PND).Results:ECG abnormalities were present in almost 2/3 of the FAP patients, whereas ECHO abnormalities occurred in around 1/3 of them. All patients with abnormal ECHO also had abnormal ECG, but the opposite did not apply. The classification tree identified ECG and ECHO as relevant variables (p < 0.001 and p = 0.08, respectively). The probability of a patient to be allocated to the PND 0 group when having a normal ECG was over 80%. When both ECG and ECHO were abnormal, this probability was null.Conclusions:Brazilian patients with FAP have frequent ECG abnormalities. ECG is an appropriate test to discriminate asymptomatic carriers of the mutation from those who develop the disease, whereas ECHO contributes to this discrimination.Sociedade Brasileira de Cardiologia - SBC2015-11-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2015002400503Arquivos Brasileiros de Cardiologia v.105 n.5 2015reponame:Arquivos Brasileiros de Cardiologia (Online)instname:Sociedade Brasileira de Cardiologia (SBC)instacron:SBC10.5935/abc.20150112info:eu-repo/semantics/openAccessQueiroz,Márcia Cavalcanti de CamposPedrosa,Roberto CouryBerensztejn,Amanda CardosoPereira,Basílio de BragançaNascimento,Emília Matos doDuarte,Martha Maria TuranoPereira-Junior,Pedro PauloCruz,Marcia Waddingtoneng2015-11-13T00:00:00Zoai:scielo:S0066-782X2015002400503Revistahttp://www.arquivosonline.com.br/https://old.scielo.br/oai/scielo-oai.php||arquivos@cardiol.br1678-41700066-782Xopendoar:2015-11-13T00:00Arquivos Brasileiros de Cardiologia (Online) - Sociedade Brasileira de Cardiologia (SBC)false |
dc.title.none.fl_str_mv |
Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
title |
Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
spellingShingle |
Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients Queiroz,Márcia Cavalcanti de Campos Amyloid Neuropathies Familial Cardiomyopathies Prealbumin Eletrocardiography |
title_short |
Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
title_full |
Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
title_fullStr |
Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
title_full_unstemmed |
Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
title_sort |
Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
author |
Queiroz,Márcia Cavalcanti de Campos |
author_facet |
Queiroz,Márcia Cavalcanti de Campos Pedrosa,Roberto Coury Berensztejn,Amanda Cardoso Pereira,Basílio de Bragança Nascimento,Emília Matos do Duarte,Martha Maria Turano Pereira-Junior,Pedro Paulo Cruz,Marcia Waddington |
author_role |
author |
author2 |
Pedrosa,Roberto Coury Berensztejn,Amanda Cardoso Pereira,Basílio de Bragança Nascimento,Emília Matos do Duarte,Martha Maria Turano Pereira-Junior,Pedro Paulo Cruz,Marcia Waddington |
author2_role |
author author author author author author author |
dc.contributor.author.fl_str_mv |
Queiroz,Márcia Cavalcanti de Campos Pedrosa,Roberto Coury Berensztejn,Amanda Cardoso Pereira,Basílio de Bragança Nascimento,Emília Matos do Duarte,Martha Maria Turano Pereira-Junior,Pedro Paulo Cruz,Marcia Waddington |
dc.subject.por.fl_str_mv |
Amyloid Neuropathies Familial Cardiomyopathies Prealbumin Eletrocardiography |
topic |
Amyloid Neuropathies Familial Cardiomyopathies Prealbumin Eletrocardiography |
description |
Background:Familial amyloidotic polyneuropathy (FAP) is a rare disease diagnosed in Brazil and worldwide. The frequency of cardiovascular involvement in Brazilian FAP patients is unknown.Objective:Detect the frequency of cardiovascular involvement and correlate the cardiovascular findings with the modified polyneuropathy disability (PND) score.Methods:In a national reference center, 51 patients were evaluated with clinical examination, electrocardiography (ECG), echocardiography (ECHO), and 24-hour Holter. Patients were classified according to the modified PND score and divided into groups: PND 0, PND I, PND II, and PND > II (which included PND IIIa, IIIb, and IV). We chose the classification tree as the statistical method to analyze the association between findings in cardiac tests with the neurological classification (PND).Results:ECG abnormalities were present in almost 2/3 of the FAP patients, whereas ECHO abnormalities occurred in around 1/3 of them. All patients with abnormal ECHO also had abnormal ECG, but the opposite did not apply. The classification tree identified ECG and ECHO as relevant variables (p < 0.001 and p = 0.08, respectively). The probability of a patient to be allocated to the PND 0 group when having a normal ECG was over 80%. When both ECG and ECHO were abnormal, this probability was null.Conclusions:Brazilian patients with FAP have frequent ECG abnormalities. ECG is an appropriate test to discriminate asymptomatic carriers of the mutation from those who develop the disease, whereas ECHO contributes to this discrimination. |
publishDate |
2015 |
dc.date.none.fl_str_mv |
2015-11-01 |
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=S0066-782X2015002400503 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2015002400503 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.5935/abc.20150112 |
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 Brasileira de Cardiologia - SBC |
publisher.none.fl_str_mv |
Sociedade Brasileira de Cardiologia - SBC |
dc.source.none.fl_str_mv |
Arquivos Brasileiros de Cardiologia v.105 n.5 2015 reponame:Arquivos Brasileiros de Cardiologia (Online) instname:Sociedade Brasileira de Cardiologia (SBC) instacron:SBC |
instname_str |
Sociedade Brasileira de Cardiologia (SBC) |
instacron_str |
SBC |
institution |
SBC |
reponame_str |
Arquivos Brasileiros de Cardiologia (Online) |
collection |
Arquivos Brasileiros de Cardiologia (Online) |
repository.name.fl_str_mv |
Arquivos Brasileiros de Cardiologia (Online) - Sociedade Brasileira de Cardiologia (SBC) |
repository.mail.fl_str_mv |
||arquivos@cardiol.br |
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1752126565717639168 |