Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type III
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Publication Date: | 2018 |
Other Authors: | , , , , |
Format: | Article |
Language: | por |
Source: | Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) |
Download full: | https://doi.org/10.29021/spdv.75.4.689 |
Summary: | Three types of hereditary angioedema (HAE) have been described within the spectrum of idioptahic nonhistaminergic angioedema. Type I and II related with C1-INH (C1-esterase inhibitor) deficiency is more frequent and type III is rare, occurs mostly in young women, without C1 inhibitor (C1-INH) alterations, and may be associated with mutations in the factor XII gene. Trauma, stress and estrogen may be the precipitants, treatment is still a challenge due to the absence of controlled studies. We present a case of possible type III HF in a young woman, aggravated by the introduction of anti-conception and improvement with its suspension and treatment with tranexamic acid, but without a family history or a factor XII mutation. |
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Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type IIIAngioedema Recidivante Idiopático: Uma Possível Apresentação de Angioedema Hereditário Tipo IIIHereditary Angioedema Type IIITranexamic AcidÁcido TranexâmicoAngioedema Hereditário Tipo IIITranexamic AcidThree types of hereditary angioedema (HAE) have been described within the spectrum of idioptahic nonhistaminergic angioedema. Type I and II related with C1-INH (C1-esterase inhibitor) deficiency is more frequent and type III is rare, occurs mostly in young women, without C1 inhibitor (C1-INH) alterations, and may be associated with mutations in the factor XII gene. Trauma, stress and estrogen may be the precipitants, treatment is still a challenge due to the absence of controlled studies. We present a case of possible type III HF in a young woman, aggravated by the introduction of anti-conception and improvement with its suspension and treatment with tranexamic acid, but without a family history or a factor XII mutation.Dentro do angioedema idiopático não histaminérgico estão descritos três tipos de angioedema hereditário (AEH). O tipo I e II por défice de C1-INH (inibidor de C1-esterase) é o mais frequente e o tipo III, mais raro, ocorre maioritariamente em mulheres jovens, sem alterações do C1-inibidor (C1-INH), e pode associar-se a mutações no gene do fator XII. Trauma, stress e estrogénios podem ser os precipitantes, o tratamento é ainda um desafio pela ausência de estudos controlados. Apresentamos um caso de possível AEH tipo III numa jovem, agravado pela introdução de contraceptivo oral e melhoria com a sua suspensão e tratamento com ácido tranexâmico, mas sem história familiar e sem mutação do factor XII.Sociedade Portuguesa de Dermatologia e Venereologia2018-01-26T00:00:00Zjournal articleinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://doi.org/10.29021/spdv.75.4.689oai:ojs.revista.spdv.com.pt:article/689Journal of the Portuguese Society of Dermatology and Venereology; Vol 75 No 4 (2017): Outubro - Dezembro; 413-416Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 75 n. 4 (2017): Outubro - Dezembro; 413-4162182-24092182-2395reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAPporhttps://revista.spdv.com.pt/index.php/spdv/article/view/689https://doi.org/10.29021/spdv.75.4.689https://revista.spdv.com.pt/index.php/spdv/article/view/689/533Sales Biermann, LaraVasconcelos Lima, IsisOliveira Santos, JulianaDantas Oliveira, PedroPassos Costa, MaiteAguiar Lima, Katiainfo:eu-repo/semantics/openAccess2022-10-06T12:35:01Zoai:ojs.revista.spdv.com.pt:article/689Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T10:31:14.388529Repositórios 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 |
Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type III Angioedema Recidivante Idiopático: Uma Possível Apresentação de Angioedema Hereditário Tipo III |
title |
Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type III |
spellingShingle |
Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type III Sales Biermann, Lara Hereditary Angioedema Type III Tranexamic Acid Ácido Tranexâmico Angioedema Hereditário Tipo III Tranexamic Acid |
title_short |
Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type III |
title_full |
Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type III |
title_fullStr |
Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type III |
title_full_unstemmed |
Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type III |
title_sort |
Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type III |
author |
Sales Biermann, Lara |
author_facet |
Sales Biermann, Lara Vasconcelos Lima, Isis Oliveira Santos, Juliana Dantas Oliveira, Pedro Passos Costa, Maite Aguiar Lima, Katia |
author_role |
author |
author2 |
Vasconcelos Lima, Isis Oliveira Santos, Juliana Dantas Oliveira, Pedro Passos Costa, Maite Aguiar Lima, Katia |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Sales Biermann, Lara Vasconcelos Lima, Isis Oliveira Santos, Juliana Dantas Oliveira, Pedro Passos Costa, Maite Aguiar Lima, Katia |
dc.subject.por.fl_str_mv |
Hereditary Angioedema Type III Tranexamic Acid Ácido Tranexâmico Angioedema Hereditário Tipo III Tranexamic Acid |
topic |
Hereditary Angioedema Type III Tranexamic Acid Ácido Tranexâmico Angioedema Hereditário Tipo III Tranexamic Acid |
description |
Three types of hereditary angioedema (HAE) have been described within the spectrum of idioptahic nonhistaminergic angioedema. Type I and II related with C1-INH (C1-esterase inhibitor) deficiency is more frequent and type III is rare, occurs mostly in young women, without C1 inhibitor (C1-INH) alterations, and may be associated with mutations in the factor XII gene. Trauma, stress and estrogen may be the precipitants, treatment is still a challenge due to the absence of controlled studies. We present a case of possible type III HF in a young woman, aggravated by the introduction of anti-conception and improvement with its suspension and treatment with tranexamic acid, but without a family history or a factor XII mutation. |
publishDate |
2018 |
dc.date.none.fl_str_mv |
2018-01-26T00:00:00Z |
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journal article info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
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publishedVersion |
dc.identifier.uri.fl_str_mv |
https://doi.org/10.29021/spdv.75.4.689 oai:ojs.revista.spdv.com.pt:article/689 |
url |
https://doi.org/10.29021/spdv.75.4.689 |
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oai:ojs.revista.spdv.com.pt:article/689 |
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por |
language |
por |
dc.relation.none.fl_str_mv |
https://revista.spdv.com.pt/index.php/spdv/article/view/689 https://doi.org/10.29021/spdv.75.4.689 https://revista.spdv.com.pt/index.php/spdv/article/view/689/533 |
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info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
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application/pdf |
dc.publisher.none.fl_str_mv |
Sociedade Portuguesa de Dermatologia e Venereologia |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Dermatologia e Venereologia |
dc.source.none.fl_str_mv |
Journal of the Portuguese Society of Dermatology and Venereology; Vol 75 No 4 (2017): Outubro - Dezembro; 413-416 Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 75 n. 4 (2017): Outubro - Dezembro; 413-416 2182-2409 2182-2395 reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia instacron:RCAAP |
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RCAAP |
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RCAAP |
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Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) |
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Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) |
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Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia |
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