Brazilian guidelines for the diagnosis and treatment of hereditary angioedema
Autor(a) principal: | |
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Data de Publicação: | 2011 |
Outros Autores: | , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Clinics |
Texto Completo: | https://www.revistas.usp.br/clinics/article/view/19435 |
Resumo: | Hereditary angioedema is an autosomal dominant disease characterized by edema attacks with multiple organ involvement. It is caused by a quantitative or functional deficiency of the C1 inhibitor, which is a member of the serine protease inhibitor family. Hereditary angioedema is unknown to many health professionals and is therefore an underdiagnosed disease. The causes of death from hereditary angioedema include laryngeal edema with asphyxia. The estimated mortality rate in patients in whom the disease goes undetected and who are therefore incorrectly treated is 25-40%. In addition to edema of the glottis, hereditary angioedema often results in edema of the gastrointestinal tract, which can be incapacitating. Patients with hereditary angioedema may undergo unnecessary surgical interventions because the digestive tract can be the primary or only organ system involved, thus mimicking acute surgical abdomen. It is estimated that patients with hereditary angioedema experience some degree of disability 20-100 days per year. The Experts in Clinical Immunology and Allergy of the "Associação Brasileira de Alergia e Imunopatologia -ASBAI" developed these guidelines for the diagnosis, therapy, and management of hereditary angioedema. |
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Brazilian guidelines for the diagnosis and treatment of hereditary angioedema Hereditary angioedemaC1 inhibitorAsphyxiaAcute surgical abdomenGuidelinesConsensus Hereditary angioedema is an autosomal dominant disease characterized by edema attacks with multiple organ involvement. It is caused by a quantitative or functional deficiency of the C1 inhibitor, which is a member of the serine protease inhibitor family. Hereditary angioedema is unknown to many health professionals and is therefore an underdiagnosed disease. The causes of death from hereditary angioedema include laryngeal edema with asphyxia. The estimated mortality rate in patients in whom the disease goes undetected and who are therefore incorrectly treated is 25-40%. In addition to edema of the glottis, hereditary angioedema often results in edema of the gastrointestinal tract, which can be incapacitating. Patients with hereditary angioedema may undergo unnecessary surgical interventions because the digestive tract can be the primary or only organ system involved, thus mimicking acute surgical abdomen. It is estimated that patients with hereditary angioedema experience some degree of disability 20-100 days per year. The Experts in Clinical Immunology and Allergy of the "Associação Brasileira de Alergia e Imunopatologia -ASBAI" developed these guidelines for the diagnosis, therapy, and management of hereditary angioedema. Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo2011-01-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://www.revistas.usp.br/clinics/article/view/1943510.1590/S1807-59322011000900021Clinics; v. 66 n. 9 (2011); 1627-1636 Clinics; Vol. 66 Núm. 9 (2011); 1627-1636 Clinics; Vol. 66 No. 9 (2011); 1627-1636 1980-53221807-5932reponame:Clinicsinstname:Universidade de São Paulo (USP)instacron:USPenghttps://www.revistas.usp.br/clinics/article/view/19435/21498Giavina-Bianchi, PedroFrança, Alfeu T.Grumach, Anete S.Motta, Abílio AFernandes, Fátima RCampos, Regis A.Valle, Solange ORosário, Nelson ASole, Dirceuinfo:eu-repo/semantics/openAccess2012-05-23T16:40:48Zoai:revistas.usp.br:article/19435Revistahttps://www.revistas.usp.br/clinicsPUBhttps://www.revistas.usp.br/clinics/oai||clinics@hc.fm.usp.br1980-53221807-5932opendoar:2012-05-23T16:40:48Clinics - Universidade de São Paulo (USP)false |
dc.title.none.fl_str_mv |
Brazilian guidelines for the diagnosis and treatment of hereditary angioedema |
title |
Brazilian guidelines for the diagnosis and treatment of hereditary angioedema |
spellingShingle |
Brazilian guidelines for the diagnosis and treatment of hereditary angioedema Giavina-Bianchi, Pedro Hereditary angioedema C1 inhibitor Asphyxia Acute surgical abdomen Guidelines Consensus |
title_short |
Brazilian guidelines for the diagnosis and treatment of hereditary angioedema |
title_full |
Brazilian guidelines for the diagnosis and treatment of hereditary angioedema |
title_fullStr |
Brazilian guidelines for the diagnosis and treatment of hereditary angioedema |
title_full_unstemmed |
Brazilian guidelines for the diagnosis and treatment of hereditary angioedema |
title_sort |
Brazilian guidelines for the diagnosis and treatment of hereditary angioedema |
author |
Giavina-Bianchi, Pedro |
author_facet |
Giavina-Bianchi, Pedro França, Alfeu T. Grumach, Anete S. Motta, Abílio A Fernandes, Fátima R Campos, Regis A. Valle, Solange O Rosário, Nelson A Sole, Dirceu |
author_role |
author |
author2 |
França, Alfeu T. Grumach, Anete S. Motta, Abílio A Fernandes, Fátima R Campos, Regis A. Valle, Solange O Rosário, Nelson A Sole, Dirceu |
author2_role |
author author author author author author author author |
dc.contributor.author.fl_str_mv |
Giavina-Bianchi, Pedro França, Alfeu T. Grumach, Anete S. Motta, Abílio A Fernandes, Fátima R Campos, Regis A. Valle, Solange O Rosário, Nelson A Sole, Dirceu |
dc.subject.por.fl_str_mv |
Hereditary angioedema C1 inhibitor Asphyxia Acute surgical abdomen Guidelines Consensus |
topic |
Hereditary angioedema C1 inhibitor Asphyxia Acute surgical abdomen Guidelines Consensus |
description |
Hereditary angioedema is an autosomal dominant disease characterized by edema attacks with multiple organ involvement. It is caused by a quantitative or functional deficiency of the C1 inhibitor, which is a member of the serine protease inhibitor family. Hereditary angioedema is unknown to many health professionals and is therefore an underdiagnosed disease. The causes of death from hereditary angioedema include laryngeal edema with asphyxia. The estimated mortality rate in patients in whom the disease goes undetected and who are therefore incorrectly treated is 25-40%. In addition to edema of the glottis, hereditary angioedema often results in edema of the gastrointestinal tract, which can be incapacitating. Patients with hereditary angioedema may undergo unnecessary surgical interventions because the digestive tract can be the primary or only organ system involved, thus mimicking acute surgical abdomen. It is estimated that patients with hereditary angioedema experience some degree of disability 20-100 days per year. The Experts in Clinical Immunology and Allergy of the "Associação Brasileira de Alergia e Imunopatologia -ASBAI" developed these guidelines for the diagnosis, therapy, and management of hereditary angioedema. |
publishDate |
2011 |
dc.date.none.fl_str_mv |
2011-01-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://www.revistas.usp.br/clinics/article/view/19435 10.1590/S1807-59322011000900021 |
url |
https://www.revistas.usp.br/clinics/article/view/19435 |
identifier_str_mv |
10.1590/S1807-59322011000900021 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
https://www.revistas.usp.br/clinics/article/view/19435/21498 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo |
publisher.none.fl_str_mv |
Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo |
dc.source.none.fl_str_mv |
Clinics; v. 66 n. 9 (2011); 1627-1636 Clinics; Vol. 66 Núm. 9 (2011); 1627-1636 Clinics; Vol. 66 No. 9 (2011); 1627-1636 1980-5322 1807-5932 reponame:Clinics instname:Universidade de São Paulo (USP) instacron:USP |
instname_str |
Universidade de São Paulo (USP) |
instacron_str |
USP |
institution |
USP |
reponame_str |
Clinics |
collection |
Clinics |
repository.name.fl_str_mv |
Clinics - Universidade de São Paulo (USP) |
repository.mail.fl_str_mv |
||clinics@hc.fm.usp.br |
_version_ |
1787713173373059072 |