» Articles » PMID: 39194031

Factors Associated with Cystic Fibrosis Mortality Before the Age of 30: Retrospective Analysis of a Cohort in Southern Brazil

Overview
Date 2024 Aug 28
PMID 39194031
Authors
Affiliations
Soon will be listed here.
Abstract

The aim of this study was to retrospectively evaluate the factors associated with mortality before the age of 30 in adults with cystic fibrosis (CF) followed up at a referral center in southern Brazil. This study included individuals over 18 years of age. Clinical data related to childhood and the period of transition to an adult healthcare of individuals with CF were recorded, as well as spirometric and mortality data of individuals between 18 and 30 years of age. A total of 48 patients were included in this study, of which 28 (58.3%) were male. Comparing groups, we observed a higher prevalence of homozygosis for the F508del mutation (P=0.028), massive hemoptysis before the age of 18 (P=0.027), and lower values of pulmonary function, forced expiratory volume in the first second (FEV1) (%) (P=0.002), forced vital capacity (FVC) (%) (P=0.01), and FEV1/FVC (%) (P=0.001) in the group that died before age 30. F508del homozygosis, episodes of massive hemoptysis in childhood, and lower FEV1 values at age 18 were related to mortality before age 30 in a cohort of individuals with CF in southern Brazil.

References
1.
Simmonds N . Ageing in cystic fibrosis and long-term survival. Paediatr Respir Rev. 2013; 14 Suppl 1:6-9. DOI: 10.1016/j.prrv.2013.01.007. View

2.
Dalcin P, de Abreu E Silva F . Cystic fibrosis in adults: diagnostic and therapeutic aspects. J Bras Pneumol. 2008; 34(2):107-17. DOI: 10.1590/s1806-37132008000200008. View

3.
Rosa K, de Lima E, Machado C, Rispoli T, Silveira V, Ongaratto R . Genetic and phenotypic traits of children and adolescents with cystic fibrosis in Southern Brazil. J Bras Pneumol. 2019; 44(6):498-504. PMC: 6459751. DOI: 10.1590/s1806-37562017000000418. View

4.
Wendekier C, Wendekier-Raybuck K . Cystic fibrosis: A changing landscape. Nurse Pract. 2021; 46(9):48-55. DOI: 10.1097/01.NPR.0000769756.82495.f3. View

5.
Keogh R, Seaman S, Barrett J, Taylor-Robinson D, Szczesniak R . Dynamic Prediction of Survival in Cystic Fibrosis: A Landmarking Analysis Using UK Patient Registry Data. Epidemiology. 2018; 30(1):29-37. PMC: 6276867. DOI: 10.1097/EDE.0000000000000920. View