Campo DC | Valor | Idioma |
dc.contributor.author | Grumach, A. S. | - |
dc.contributor.author | Valle, S. O. R. | - |
dc.contributor.author | Toledo, E. | - |
dc.contributor.author | Vasconcelos, D. de Moraes | - |
dc.contributor.author | Villela, M. M. S. | - |
dc.contributor.author | Mansour, E. | - |
dc.contributor.author | Pinto, J. A. | - |
dc.contributor.author | Campos, Régis de Albuquerque | - |
dc.contributor.author | França, A. T. | - |
dc.creator | Grumach, A. S. | - |
dc.creator | Valle, S. O. R. | - |
dc.creator | Toledo, E. | - |
dc.creator | Vasconcelos, D. de Moraes | - |
dc.creator | Villela, M. M. S. | - |
dc.creator | Mansour, E. | - |
dc.creator | Pinto, J. A. | - |
dc.creator | Campos, Régis de Albuquerque | - |
dc.creator | França, A. T. | - |
dc.date.accessioned | 2013-11-22T12:07:15Z | - |
dc.date.issued | 2013 | - |
dc.identifier.issn | 0926-9959 | - |
dc.identifier.uri | http://repositorio.ufba.br/ri/handle/ri/13842 | - |
dc.description | Texto completo. Acesso restrito. p. 338-344 | pt_BR |
dc.description.abstract | Background Hereditary Angio-oedema (HAE) is a serious medical condition caused by a rare autosomal dominant
genetic disorder, in which C1 inhibitor (C1-INH) function is reduced. There is no organized information on the HAE
patient population in Brazil.
Objective The Brazilian Registry was established to disseminate diagnostic access, and to better understand the
main features of the disease in our country and its clinical impact.
Methods A questionnaire was prepared and sent to specialists. The completed questionnaires were forwarded to
the coordinating site and then entered into the Registry. Samples from patients with an unconfirmed diagnosis were
tested for C1 inhibitor and C4 levels.
Results From 2006 to 2010, 210 patients (133 females; mean age, 30 ±17 years) were included. The median age
of onset of symptoms and age at diagnosis were 6.5 and 21 years, respectively; 80.9% of the patients had
subcutaneous oedema, 54% gastrointestinal and 35.7% respiratory symptoms (21% had laryngeal oedema).
Laparotomy due to the disease was performed in 6.2% of the patients. The majority of patients had Type I HAE of
moderate severity. Twenty-seven per cent did not receive treatment; 53% were treated with danazol alone.
Conclusion A paucity of patients with Type II HAE and a high frequency of laparotomy were observed, highlighting
the need for better diagnosis in Brazil. HAE related educational activities, improved diagnosis and access to
available therapy are needed in Brazil. | pt_BR |
dc.language.iso | en | pt_BR |
dc.publisher | Journal of the European Academy of Dermatology and Venereology | pt_BR |
dc.rights | Acesso Aberto | pt_BR |
dc.source | 10.1111/j.1468-3083.2012.04670.x | pt_BR |
dc.title | Hereditary angioedema: first report of the Brazilian registry and challenges | pt_BR |
dc.title.alternative | Journal of the European Academy of Dermatology and Venereology | pt_BR |
dc.type | Artigo de Periódico | pt_BR |
dc.description.localpub | Salvador | pt_BR |
dc.identifier.number | v. 27, n. 3 | pt_BR |
dc.embargo.liftdate | 10000-01-01 | - |
Aparece nas coleções: | Artigo Publicado em Periódico (Faculdade de Medicina)
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