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

Overview

Journal Diagnostics (Basel)

Specialty Radiology

Date 2023 Feb 25

PMID 36832251

Authors

Luan Victor Frota de Azevedo

Fernanda Cristine Ribeiro Medeiros Cruz

Jessica Paula Martins

Fernando Augusto Lima Marson

Affiliations

Soon will be listed here.

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/) 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 ( = 1492; 48.9%) and female ( = 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.

Citing Articles

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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.

Marques L, Boschiero M, Sansone N, Brienze L, Marson F Healthcare (Basel). 2023; 11(13).

PMID: 37444770 PMC: 10341451. DOI: 10.3390/healthcare11131936.

References

Montemayor K, Jain R . Cystic Fibrosis: Highly Effective Targeted Therapeutics and the Impact on Sex and Racial Disparities. Med Clin North Am. 2022; 106(6):1001-1012. DOI: 10.1016/j.mcna.2022.07.005. View

Palamim C, Boschiero M, Valencise F, Marson F . Human Development Index Is Associated with COVID-19 Case Fatality Rate in Brazil: An Ecological Study. Int J Environ Res Public Health. 2022; 19(9). PMC: 9102208. DOI: 10.3390/ijerph19095306. View

Scotet V, LHostis C, Ferec C . The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the Gene Discovery. Genes (Basel). 2020; 11(6). PMC: 7348877. DOI: 10.3390/genes11060589. View

Silva Filho L, Castanos C, Ruiz H . Cystic fibrosis in Latin America-Improving the awareness. J Cyst Fibros. 2016; 15(6):791-793. DOI: 10.1016/j.jcf.2016.05.007. View

Guo J, Garratt A, Hill A . Worldwide rates of diagnosis and effective treatment for cystic fibrosis. J Cyst Fibros. 2022; 21(3):456-462. DOI: 10.1016/j.jcf.2022.01.009. View

McGarry M, Williams 2nd W, McColley S . The demographics of adverse outcomes in cystic fibrosis. Pediatr Pulmonol. 2019; 54 Suppl 3:S74-S83. PMC: 6857719. DOI: 10.1002/ppul.24434. View

Athanazio R, Silva Filho L, Vergara A, Ribeiro A, Riedi C, Procianoy E . Brazilian guidelines for the diagnosis and treatment of cystic fibrosis. J Bras Pneumol. 2017; 43(3):219-245. PMC: 5687954. DOI: 10.1590/S1806-37562017000000065. View

Hutchins K, Barr E, Bellcross C, Ali N, Hunt W . Evaluating Differences in the Disease Experiences of Minority Adults With Cystic Fibrosis. J Patient Exp. 2022; 9:23743735221112629. PMC: 9289912. DOI: 10.1177/23743735221112629. View

Kmit A, Marson F, Pereira S, Vinagre A, Leite G, Servidoni M . Extent of rescue of F508del-CFTR function by VX-809 and VX-770 in human nasal epithelial cells correlates with SNP rs7512462 in SLC26A9 gene in F508del/F508del Cystic Fibrosis patients. Biochim Biophys Acta Mol Basis Dis. 2019; 1865(6):1323-1331. DOI: 10.1016/j.bbadis.2019.01.029. View

10.

Marson F . Cost of precision medicine at a referral center for cystic fibrosis. J Bras Pneumol. 2020; 46(2):e20190308. PMC: 7462696. DOI: 10.36416/1806-3756/e20190308. View

11.

Vaidyanathan S, Trumbull A, Bar L, Rao M, Yu Y, Sellers Z . CFTR genotype analysis of Asians in international registries highlights disparities in the diagnosis and treatment of Asian patients with cystic fibrosis. Genet Med. 2022; 24(10):2180-2186. DOI: 10.1016/j.gim.2022.06.009. View

12.

Marson F, Bertuzzo C, Ribeiro J . Classification of CFTR mutation classes. Lancet Respir Med. 2016; 4(8):e37-e38. DOI: 10.1016/S2213-2600(16)30188-6. View

13.

Sansone N, Boschiero M, Valencise F, Palamim C, Marson F . Characterization of demographic data, clinical signs, comorbidities, and outcomes according to the race in hospitalized individuals with COVID-19 in Brazil: An observational study. J Glob Health. 2022; 12:05027. PMC: 9309002. DOI: 10.7189/jogh.12.05027. View

14.

Servidoni M, Souza Gomez C, Marson F, Toro A, Goncalves de Oliveira Ribeiro M, Ribeiro J . Sweat test and cystic fibrosis: overview of test performance at public and private centers in the state of São Paulo, Brazil. J Bras Pneumol. 2017; 43(2):121-128. PMC: 5474375. DOI: 10.1590/S1806-37562016000000076. View

15.

Schrijver I, Pique L, Graham S, Pearl M, Cherry A, Kharrazi M . The Spectrum of CFTR Variants in Nonwhite Cystic Fibrosis Patients: Implications for Molecular Diagnostic Testing. J Mol Diagn. 2015; 18(1):39-50. DOI: 10.1016/j.jmoldx.2015.07.005. View

16.

Stewart C, Pepper M . Cystic Fibrosis in the African Diaspora. Ann Am Thorac Soc. 2016; 14(1):1-7. DOI: 10.1513/AnnalsATS.201606-481FR. View

17.

Zemanick E, Taylor-Cousar J, Davies J, Gibson R, Mall M, McKone E . A Phase 3 Open-Label Study of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One Allele. Am J Respir Crit Care Med. 2021; 203(12):1522-1532. PMC: 8483230. DOI: 10.1164/rccm.202102-0509OC. View

18.

Dagenais R, Su V, Quon B . Real-World Safety of CFTR Modulators in the Treatment of Cystic Fibrosis: A Systematic Review. J Clin Med. 2020; 10(1). PMC: 7795777. DOI: 10.3390/jcm10010023. View

19.

Silva Filho L, Marostica P, Athanazio R, Reis F, Damaceno N, Paes A . Extensive CFTR sequencing through NGS in Brazilian individuals with cystic fibrosis: unravelling regional discrepancies in the country. J Cyst Fibros. 2020; 20(3):473-484. DOI: 10.1016/j.jcf.2020.08.007. View

20.

Pereira S, Ribeiro J, Ribeiro A, Bertuzzo C, Marson F . Novel, rare and common pathogenic variants in the CFTR gene screened by high-throughput sequencing technology and predicted by in silico tools. Sci Rep. 2019; 9(1):6234. PMC: 6470152. DOI: 10.1038/s41598-019-42404-6. View