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Title: Brazilian guidelines for the diagnosis and treatment of hereditary angioedema
Authors: Giavina-Bianchi, Pedro
Franca, Alfeu T.
Grumach, Anete S.
Motta, Abilio A.
Fernandes, Fatima R.
Campos, Regis A.
Valle, Solange O.
Rosario, Nelson A.
Solé, Dirceu [UNIFESP]
Universidade de São Paulo (USP)
Universidade Federal do Rio de Janeiro (UFRJ)
Hosp Servidor Publ Estadual São Paulo FMO
Universidade Federal da Bahia (UFBA)
Univ Fed Parana
Universidade Federal de São Paulo (UNIFESP)
Keywords: Hereditary angioedema
C1 inhibitor
Acute surgical abdomen
Issue Date: 1-Jan-2011
Publisher: Hospital Clinicas, Univ São Paulo
Citation: Clinics. São Paulo: Hospital Clinicas, Univ São Paulo, v. 66, n. 9, p. 1627-1636, 2011.
Abstract: 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.
ISSN: 1807-5932
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