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

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 São 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 Boton Pereira et al. Epidemiologic, Clinical, Geospatial Approach to PID 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.

Double Trouble: Dengue Followed by COVID-19 Infection Acquired in Two Different Regions: A Doctor’s Case Report and Spatial Distribution of Cases in Presidente Prudente, São Paulo, Brazil

“…The state of São Paulo is divided geographically into 15 mesoregions and 18 Regional Networks for Health Assistance (RNHAs). The western region harbors 45 municipalities and is administered by RNHA11, located in Presidente Prudente, the biggest and main city of mesoregion 8 [17]. The city is a mid-sized urban center about 560 km from the state capital, São Paulo.…”

Clinical, Epidemiological, and Geospatial Characteristics of Patients Infected with Hepatitis C Virus Treated with Second-Generation Direct-Action Antivirals in a Reference Center in a Mesoregion of São Paulo State, Brazil

“…Geographically, the state is divided into 15 mesoregions and 18 regional health care networks (RHCNs) for administrative health care. The western region of São Paulo state, with 45 municipalities and an estimated population of 753,344 in 2018 (IBGE), is considered to be one of the poorest and asymmetric regions of São Paulo state [ 13 ]. The region is characterized by small municipalities with <20,000 inhabitants.…”

“…The city is a mid-sized urban center 560 km from the state capital, São Paulo. In 2019, the estimated population was 228,743 inhabitants (IBGE) [ 13 ]. The Regional Hospital of Presidente Prudente (RH), located in Presidente Prudente, is a 550-bed tertiary-care public university hospital providing care for SUS patients, and since 2000, it has been a reference center for the diagnosis and treatment of viral hepatitis caused by HBV and HCV [ 14 ].…”

“…In epidemiologic and public health studies, geospatial analysis has been used successfully for vector-borne diseases regarding environmental health, risk assessment, and injury prediction, and for the development of health policies and intervention planning [ 12 , 13 ]. In Brazil, clinical and epidemiologic studies on individuals chronically infected with HBV and HCV showed that capitals and large cities are the focus of infection in specific vulnerable groups.…”

Cases and distribution of visceral leishmaniasis in western São Paulo: A neglected disease in this region of Brazil

“…The Pontal of Paranapanema covers an area of 18,392.16 km 2 and has an estimated population of 620,953 [8] (Fig 1A). In contrast to the dynamic economic situation in the rest of the state, it is considered one of the poorest regions of São Paulo state [9]. Local characteristics.…”