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Practice Guideline
. 2018:73:e310.
doi: 10.6061/clinics/2018/e310. Epub 2018 May 3.

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

Pedro Giavina-Bianchi  1 , Luisa Karla Arruda  2 , Marcelo V Aun  1 , Regis A Campos  3 , Herberto J Chong-Neto  4 , Rosemeire N Constantino-Silva  5 , Fátima R Fernandes  6 , Maria F Ferraro  2 , Mariana P L Ferriani  2 , Alfeu T França  7 , Gustavo Fusaro  8 , Juliana F B Garcia  1 , Shirley Komninakis  5 , Luana S M Maia  2 , Eli Mansour  9 , Adriana S Moreno  2 , Antonio A Motta  1 , João B Pesquero  10 , Nathalia Portilho  1 , Nelson A Rosário  4 , Faradiba S Serpa  11 , Dirceu Solé  12 , Priscila Takejima  1 , Eliana Toledo  13 , Solange O.R Valle  7 , Camila L Veronez  10 , Anete S Grumach  5
Affiliations
Practice Guideline

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

Pedro Giavina-Bianchi et al. Clinics (Sao Paulo). 2018.

Abstract

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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Figures

Figure 1
Figure 1
Kallikrein-kinin system.
Figure 2
Figure 2
A: Warning Signs. B: HAAAAE for Heredity, recurrent Angioedema, recurrent Abdominal pain, Absence of urticaria, Absence of response to antihistamines and association with Estrogen.
Figure 3
Figure 3
Algorithm of HAE diagnosis.
Figure 4
Figure 4
Classification of Angioedema by Endotypes ,.
See this image and copyright information in PMC

References

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