Clinics
Autor: | Bianchi, Pedro Giavina, França, Alfeu T., Grumach, Anete S., Motta, Abílio A., Fernandes, Fátima R., Campos, Régis de Albuquerque, Valle, Solange O., Rosário, Nelson A., Solé, Dirceu |
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Jazyk: | angličtina |
Rok vydání: | 2011 |
Předmět: | |
Zdroj: | Repositório Institucional da UFBA Universidade Federal da Bahia (UFBA) instacron:UFBA |
DOI: | 10.1590/S1807-59322011000900021 |
Popis: | p. 1627-1636 Submitted by Edileide Reis (leyde-landy@hotmail.com) on 2014-02-19T13:43:29Z No. of bitstreams: 1 Regis A. Campos.pdf: 254414 bytes, checksum: db4489a3c989b1cb781cd78756cb885c (MD5) Approved for entry into archive by Flávia Ferreira (flaviaccf@yahoo.com.br) on 2015-10-13T12:28:26Z (GMT) No. of bitstreams: 1 Regis A. Campos.pdf: 254414 bytes, checksum: db4489a3c989b1cb781cd78756cb885c (MD5) Made available in DSpace on 2015-10-13T12:28:26Z (GMT). No. of bitstreams: 1 Regis A. Campos.pdf: 254414 bytes, checksum: db4489a3c989b1cb781cd78756cb885c (MD5) Previous issue date: 2011 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 "Associação Brasileira de Alergia e Imunopatologia -ASBAI" developed these guidelines for the diagnosis, therapy, and management of hereditary angioedema. |
Databáze: | OpenAIRE |
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