Idiopathic Recurring Angioedema: A Possible Case of Hereditary Angioedema Type III

Bibliographic Details
Main Author: Sales Biermann, Lara
Publication Date: 2018
Other Authors: Vasconcelos Lima, Isis, Oliveira Santos, Juliana, Dantas Oliveira, Pedro, Passos Costa, Maite, Aguiar Lima, Katia
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.
id RCAP_1b3bc5e4d2bd68b758695ba331e331d7
oai_identifier_str oai:ojs.revista.spdv.com.pt:article/689
network_acronym_str RCAP
network_name_str Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
repository_id_str https://opendoar.ac.uk/repository/7160
spelling 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
dc.type.driver.fl_str_mv journal article
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 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
identifier_str_mv oai:ojs.revista.spdv.com.pt:article/689
dc.language.iso.fl_str_mv 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
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 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
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
_version_ 1833590939962048512