Social-environmental phenotypes of rapid cystic fibrosis lung disease progression in adolescents and young adults living in the United States.

Autor:	Palipana AK; Duke University, Durham, NC, United States.; Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States., Vancil A; Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States., Gecili E; Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.; Department of Pediatrics, University of Cincinnati, Cincinnati, OH, United States., Rasnick E; Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States., Ehrlich D; Duke University, Durham, NC, United States.; Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States., Pestian T; Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States., Andrinopoulou ER; Department of Biostatistics, Erasmus Medical Center, Rotterdam, the Netherlands.; Department of Epidemiology, Erasmus Medical Center, Rotterdam, the Netherlands., Afonso PM; Department of Biostatistics, Erasmus Medical Center, Rotterdam, the Netherlands.; Department of Epidemiology, Erasmus Medical Center, Rotterdam, the Netherlands., Keogh RH; London School of Hygiene and Tropical Medicine, London, UK., Ni Y; Kaiser Permanente, Denver, CO, United States., Dexheimer JW; Department of Pediatrics, University of Cincinnati, Cincinnati, OH, United States.; Division of Biomedical Informatics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States., Clancy JP; Cystic Fibrosis Foundation, Bethesda, MD, United States., Ryan P; Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.; Department of Pediatrics, University of Cincinnati, Cincinnati, OH, United States., Brokamp C; Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.; Department of Pediatrics, University of Cincinnati, Cincinnati, OH, United States., Szczesniak RD; Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.; Department of Pediatrics, University of Cincinnati, Cincinnati, OH, United States.; Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
Jazyk:	angličtina
Zdroj:	Environmental advances [Environ Adv] 2023 Dec; Vol. 14. Date of Electronic Publication: 2023 Nov 10.
DOI:	10.1016/j.envadv.2023.100449
Abstrakt:	Background: Cystic fibrosis (CF) is a genetic disease but is greatly impacted by non-genetic (social/environmental and stochastic) influences. Some people with CF experience rapid decline, a precipitous drop in lung function relative to patient- and/or center-level norms. Those who experience rapid decline in early adulthood, compared to adolescence, typically exhibit less severe clinical disease but greater loss of lung function. The extent to which timing and degree of rapid decline are informed by social and environmental determinants of health (geomarkers) is unknown. Methods: A longitudinal cohort study was performed (24,228 patients, aged 6-21 years) using the U.S. CF Foundation Patient Registry. Geomarkers at the ZIP Code Tabulation Area level measured air pollution/respiratory hazards, greenspace, crime, and socioeconomic deprivation. A composite score quantifying social-environmental adversity was created and used in covariate-adjusted functional principal component analysis, which was applied to cluster longitudinal lung function trajectories. Results: Social-environmental phenotyping yielded three primary phenotypes that corresponded to early, middle, and late timing of peak decline in lung function over age. Geographic differences were related to distinct cultural and socioeconomic regions. Extent of peak decline, estimated as forced expiratory volume in 1 s of % predicted/year, ranged from 2.8 to 4.1 % predicted/year depending on social-environmental adversity. Middle decliners with increased social-environmental adversity experienced rapid decline 14.2 months earlier than their counterparts with lower social-environmental adversity, while timing was similar within other phenotypes. Early and middle decliners experienced mortality peaks during early adolescence and adulthood, respectively. Conclusion: While early decliners had the most severe CF lung disease, middle and late decliners lost more lung function. Higher social-environmental adversity associated with increased risk of rapid decline and mortality during young adulthood among middle decliners. This sub-phenotype may benefit from enhanced lung-function monitoring and personalized secondary environmental health interventions to mitigate chemical and non-chemical stressors. Competing Interests: Declaration of Competing Interest Author RDS serves on the Cystic Fibrosis Foundation Patient Registry Committee. The remaining authors have no conflicts of interest to report.
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu