Primary Immunodeficiencies in a Mesoregion of Sao Paulo, Brazil: Epidemiologic, Clinical, and Geospatial Approach

Registro completo de metadados
MetadadosDescriçãoIdioma
Autor(es): dc.contributorReg Hosp Presidente Prudente-
Autor(es): dc.contributorOeste Paulista Univ-
Autor(es): dc.contributorUniversidade Estadual Paulista (Unesp)-
Autor(es): dc.contributorUniversidade de São Paulo (USP)-
Autor(es): dc.creatorBoton Pereira, Denise Helena-
Autor(es): dc.creatorPrimo, Livia Souza-
Autor(es): dc.creatorPelizari, Giovana-
Autor(es): dc.creatorFlores, Edilson [UNESP]-
Autor(es): dc.creatorMoraes-Vasconcelos, Dewton de-
Autor(es): dc.creatorCondino-Neto, Antonio-
Autor(es): dc.creatorPrestes-Carneiro, Luiz Euribel-
Data de aceite: dc.date.accessioned2022-02-22T00:10:50Z-
Data de disponibilização: dc.date.available2022-02-22T00:10:50Z-
Data de envio: dc.date.issued2020-12-09-
Data de envio: dc.date.issued2020-12-09-
Data de envio: dc.date.issued2020-05-12-
Fonte completa do material: dc.identifierhttp://dx.doi.org/10.3389/fimmu.2020.00862-
Fonte completa do material: dc.identifierhttp://hdl.handle.net/11449/196931-
Fonte: dc.identifier.urihttp://educapes.capes.gov.br/handle/11449/196931-
Descrição: dc.descriptionBackground: Primary immunodeficiencies (PIDs) are rare genetic disorders leading to immunologic abnormalities that can affect different organs and systems. We determined the epidemiology, clinical, and geospatial characteristics of PID disorders among patients diagnosed over a 5 year period in a reference hospital covering a mesoregion in Sao Paulo, Brazil. Methods: A retrospective analysis of 39 patients with recognizable PIDs according to the criteria of the European Society of Primary Immunodeficiencies were enrolled. Thirty-four patients came from outpatient immunodeficiency clinics and five patients from active search. Demographic, clinical, and immunologic data were collected, and maps were constructed using a geographic information system. Results: The ratio of females to males was 1.4:1, and 48.7% of patients were younger than 17 years of age. The mean age at the onset of symptoms in children was 2.0 years [standard error of the mean (SEM), 1.7 years] and the diagnosis lag was 5.1 years (SEM, 3.1 years); the mean age at diagnosis in adults was 16.3 years (SEM, 11.8 years) and the lag was 10.8 years (SEM, 10.9 years). Antibody deficiency and common variable immunodeficiencies were the most common categories and phenotypes, respectively. The need for intravenous antibiotics and respiratory tract infections were the most prevalent warning signs, with an overall mortality rate of 15.3%. Autoimmune diseases were diagnosed in 56.4% and visceral leishmaniasis in 5.1% of patients. In the active search, 29 patients were investigated and 17.2% were diagnosed; early diagnosis, the involvement of multidisciplinary professionals, and dissemination of knowledge achieved milestone benefits. The distribution of PID networks in Brazil shows great asymmetry between regions and at a regional level; it was shown that the patients lived mainly in Presidente Prudente municipality. Conclusions: The implementation of an immunodeficiency outpatient clinic in a referral hospital covering a mesoregion with a large population has led to the generation of policies and practices to improve the diagnosis, quality of life, and care of patients with PIDs and their families. Furthermore, the search for hospitalized patients with warning signs for PIDs showed great benefits. Inequality in the distribution of PID network centers in Brazil was demonstrated.-
Descrição: dc.descriptionFundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)-
Descrição: dc.descriptionReg Hosp Presidente Prudente, Imunnodeficiencies Outpatient Clin, Presidente Prudente, Brazil-
Descrição: dc.descriptionOeste Paulista Univ, Dept Pediat, Presidente Prudente, Brazil-
Descrição: dc.descriptionUniv Estadual Paulista, Dept Stat, Presidente Prudente, Brazil-
Descrição: dc.descriptionUniv Sao Paulo, Fac Med, Hosp Clin, Lab Med Invest Unit 56, Sao Paulo, Brazil-
Descrição: dc.descriptionUniv Sao Paulo, Dept Immunol, Inst Biomed Sci, Sao Paulo, Brazil-
Descrição: dc.descriptionOeste Paulista Univ, Dept Internal Med, Presidente Prudente, Brazil-
Descrição: dc.descriptionUniv Estadual Paulista, Dept Stat, Presidente Prudente, Brazil-
Descrição: dc.descriptionFAPESP: 2018/17522-3-
Formato: dc.format11-
Idioma: dc.languageen-
Publicador: dc.publisherFrontiers Media Sa-
Relação: dc.relationFrontiers In Immunology-
???dc.source???: dc.sourceWeb of Science-
Palavras-chave: dc.subjectprimary immunodeficiencies-
Palavras-chave: dc.subjectactive search-
Palavras-chave: dc.subjectantibody deficiency-
Palavras-chave: dc.subjectautoimmune diseases-
Palavras-chave: dc.subjectmesoregion-
Palavras-chave: dc.subjectepidemiology-
Palavras-chave: dc.subjectgeospatial-
Título: dc.titlePrimary Immunodeficiencies in a Mesoregion of Sao Paulo, Brazil: Epidemiologic, Clinical, and Geospatial Approach-
Tipo de arquivo: dc.typelivro digital-
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