Brazilian guidelines for the diagnosis and treatment of hereditary angioedema

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art_GIAVINA-BIANCHI_Brazilian_guidelines_for_the_diagnosis_and_treatment_of_2011.PDF (248.45 KB)
Citações na Scopus
21
Tipo de produção
article
Data de publicação
2011
Editora
HOSPITAL CLINICAS, UNIV SAO PAULO
DOI
Indexadores
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Autores
FRANCA, Alfeu T.
FERNANDES, Fatima R.
CAMPOS, Regis A.
VALLE, Solange O.
ROSARIO, Nelson A.
SOLE, Dirceu
Autor de Grupo de pesquisa
Editores
Coordenadores
Organizadores
Citação
CLINICS, v.66, n.9, p.1627-1636, 2011
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Hereditary angioedema is an autosomal dominant disease characterized by edema attacks with multiple organ involvement. It is caused by a quantitative or functional deficiency of the C1 inhibitor, which is a member of the serine protease inhibitor family. Hereditary angioedema is unknown to many health professionals and is therefore an underdiagnosed disease. The causes of death from hereditary angioedema include laryngeal edema with asphyxia. The estimated mortality rate in patients in whom the disease goes undetected and who are therefore incorrectly treated is 25-40%. In addition to edema of the glottis, hereditary angioedema often results in edema of the gastrointestinal tract, which can be incapacitating. Patients with hereditary angioedema may undergo unnecessary surgical interventions because the digestive tract can be the primary or only organ system involved, thus mimicking acute surgical abdomen. It is estimated that patients with hereditary angioedema experience some degree of disability 20-100 days per year. The Experts in Clinical Immunology and Allergy of the ""Associacao Brasileira de Alergia e Imunopatologia - ASBAI"" developed these guidelines for the diagnosis, therapy, and management of hereditary angioedema.
Palavras-chave
Hereditary angioedema, C1 inhibitor, Asphyxia, Acute surgical abdomen, Guidelines, Consensus
URI
https://observatorio.fm.usp.br/handle/OPI/22573
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Coleções
Artigos e Materiais de Revistas Científicas - FM/MCM
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - LIM/60