Brazilian Guidelines for Hereditary Angioedema Management - 2017 Update Part 1: Definition, Classification and Diagnosis

Hereditary angioedema is an autosomal dominant disease characterized by recurrent angioedema attacks with the involvement of multiple organs. The disease is unknown to many health professionals and is therefore underdiagnosed. Patients who are not adequately diagnosed and treated have an estimated m...

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Detalles Bibliográficos
Autores principales: Giavina-Bianchi, Pedro, Arruda, Luisa Karla, Aun, Marcelo V., Campos, Regis A., Chong-Neto, Herberto J., Constantino-Silva, Rosemeire N., Fernandes, Fátima R., Ferraro, Maria F., Ferriani, Mariana P.L., França, Alfeu T., Fusaro, Gustavo, Garcia, Juliana F.B., Komninakis, Shirley, Maia, Luana S.M., Mansour, Eli, Moreno, Adriana S., Motta, Antonio A., Pesquero, João B., Portilho, Nathalia, Rosário, Nelson A., Serpa, Faradiba S., Solé, Dirceu, Takejima, Priscila, Toledo, Eliana, Valle, Solange O.R., Veronez, Camila L., Grumach, Anete S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo 2018
Materias:
Review Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5910635/
https://www.ncbi.nlm.nih.gov/pubmed/29723342
http://dx.doi.org/10.6061/clinics/2018/e310
Descripción
Sumario:Hereditary angioedema is an autosomal dominant disease characterized by recurrent angioedema attacks with the involvement of multiple organs. The disease is unknown to many health professionals and is therefore underdiagnosed. Patients who are not adequately diagnosed and treated have an estimated mortality rate ranging from 25% to 40% due to asphyxiation by laryngeal angioedema. Intestinal angioedema is another important and incapacitating presentation that may be the main or only manifestation during an attack. In this article, a group of experts from the “Associação Brasileira de Alergia e Imunologia (ASBAI)” and the “Grupo de Estudos Brasileiro em Angioedema Hereditário (GEBRAEH)” has updated the Brazilian guidelines for the diagnosis and treatment of hereditary angioedema.

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