| Full text | |
| Author(s): |
Boton Pereira, Denise Helena
[1]
;
Primo, Livia Souza
[1]
;
Pelizari, Giovana
[2]
;
Flores, Edilson
[3]
;
de Moraes-Vasconcelos, Dewton
[4]
;
Condino-Neto, Antonio
[5]
;
Prestes-Carneiro, Luiz Euribel
[1, 6]
Total Authors: 7
|
| Affiliation: | [1] Reg Hosp Presidente Prudente, Imunnodeficiencies Outpatient Clin, Presidente Prudente - Brazil
[2] Oeste Paulista Univ, Dept Pediat, Presidente Prudente - Brazil
[3] Univ Estadual Paulista, Dept Stat, Presidente Prudente - Brazil
[4] Univ Sao Paulo, Fac Med, Hosp Clin, Lab Med Invest Unit 56, Sao Paulo - Brazil
[5] Univ Sao Paulo, Dept Immunol, Inst Biomed Sci, Sao Paulo - Brazil
[6] Oeste Paulista Univ, Dept Internal Med, Presidente Prudente - Brazil
Total Affiliations: 6
|
| Document type: | Journal article |
| Source: | FRONTIERS IN IMMUNOLOGY; v. 11, MAY 12 2020. |
| Web of Science Citations: | 0 |
| Abstract | |
Background: 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. (AU) | |
| FAPESP's process: | 18/17522-3 - Classification and active search of patients with primary immunodeficiency in a reference Public Hospital of São Paulo state's western region |
| Grantee: | Lívia Souza Primo |
| Support Opportunities: | Scholarships in Brazil - Scientific Initiation |