Cystic Fibrosis: A Descriptive Analysis of Deaths in a Two-Decade Period in Brazil According to Age, Race, and Sex

Abstract

The diagnosis of cystic fibrosis has improved in the last few years due to greater access to diagnostic tools and the evolution of molecular biology; the knowledge obtained has contributed to the understanding of its death profile. In this context, an epidemiological study was developed focusing on deaths from cystic fibrosis in Brazil from 1996 to 2019. The data were collected from the Data-SUS (Unified National Health System Information Technology Department from Brazil). The epidemiological analysis included patients’ age groups, racial groups, and sex. In our data, between 1996 and 2019, Σ3050 deaths were recorded, totaling a ≅330% increase in the number of deaths resulting from cystic fibrosis. This fact might be related to a better diagnosis of the disease, mainly in patients from racial groups that are not commonly associated with cystic fibrosis, such as Black individuals, Hispanic or Latino (mixed individuals/Pardos) individuals, and American Indians (Indigenous peoples from Brazil). Regarding of race, the Σ of deaths was: nine (0.3%) in the American Indian group, 12 (0.4%) in the Asian group, 99 (3.6%) in the Black or African American group, 787 (28.6%) in the Hispanic or Latino group, and 1843 (67.0%) in the White group. The White group showed the highest prevalence of deaths, and the increase in mortality was ≅150 times in this group, while, in the Hispanic or Latino group, it was ≅75 times. Regarding sex, the numbers and percentage of deaths of both male (N = 1492; 48.9%) and female (N = 1557; 51.1%) patients were seen to be relatively close. As for age groups, the >60-year-old group presented the most significant results, with an increase of ≅60 times in the registered deaths. In conclusion, in Brazil, despite the number of deaths from cystic fibrosis being prevalent in the White group, it increased in all racial groups (Hispanic or Latino, Black or African American, American Indian, or Asian individuals) and was associated with older age.

Overview of childhood vaccination coverage in Brazil and the impact of the COVID-19 pandemic: Is our children’s health at risk? A review of pre-COVID-19 periods and during the COVID-19 pandemic

“…An alpha error of 0.05 was employed in the statistical analysis. Our research group used a statistical approach similar to that used in two previous studies, namely, one of them associated the number of deaths of patients undergoing hospital treatment for acute respiratory syndrome caused by COVID-19 in Brazil and the HDI, while the other associated the number of deaths due to the clinical evolution of cystic fibrosis [Online Mendelian Inheritance in Man (OMIM) no 219700] and HDI [37] , [38] .…”

Epidemiological Profile of Hospitalized Patients with Cystic Fibrosis in Brazil Due to Severe Acute Respiratory Infection during the COVID-19 Pandemic and a Systematic Review of Worldwide COVID-19 in Those with Cystic Fibrosis

“…219700), one of the most common Mendelian autosomal recessive disorders, is characterized by two aberrant copies of the Cystic Fibrosis Transmembrane Conductance Regulator ( CFTR ; region 7q31.2) gene [ 1 ], which ultimately alter the CFTR protein, an ion channel responsible for transporting chloride and bicarbonate across epithelial surfaces, leading to the dysregulation of endothelial cells and decreased pH in airway surface liquid [ 1 , 2 , 3 ]. Most CF symptoms are related to the respiratory tract, such as mucus accumulation and difficulty cleaning the respiratory tract, which could lead to an enhanced risk of chronic infections, inflammation [ 2 , 3 ], and death [ 4 ].…”