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...

Full description

Bibliographic Details
Authors: Giavina-Bianchi, Pedro, Arruda, Luisa Karla, Aun, Marcelo V., Campos, Regis A., Chong-Neto, Herberto J., Constantino-Silva, Rosemeire N., Fernandes, Fatima R., Ferraro, Maria F., Ferriani, Mariana P. L., Franca, Alfeu T., Fusaro, Gustavo, Garcia, Juliana F. B., Komninakis, Shirley, Maia, Luana S. M., Mansour, Eli, Moreno, Adriana S., Motta, Antonio A., Pesquero, Joao B. [UNIFESP], Portilho, Nathalia, Rosario, Nelson A., Serpa, Faradiba S., Sole, Dirceu, Takejima, Priscila, Toledo, Eliana, Valle, Solange O. R., Veronez, Camila L. [UNIFESP], Grumach, Anete S.
Format: article
Status:Published version
Publication Date:2018
Country:Brasil
Institution:Universidade Federal de São Paulo (UNIFESP)
Repository:Repositório Institucional da UNIFESP
Language:English
OAI Identifier:oai:repositorio.unifesp.br:11600/54325
Online Access:http://dx.doi.org/10.6061/clinics/2018/e310
https://repositorio.unifesp.br/handle/11600/54325
Access Level:Open access
Keyword:Hereditary Angioedema
Angioedema
C1 Inhibitor Deficiency
Coagulation Factor XII Mutations
Management
Guidelines
Description
Summary: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 "Associacao Brasileira de Alergia e Imunologia (ASBAI)'' and the "Grupo de Estudos Brasileiro em Angioedema Hereditario (GEBRAEH)'' has updated the Brazilian guidelines for the diagnosis and treatment of hereditary angioedema.

Similar Items