Indoor air pollution exposure is associated with greater morbidity in cystic fibrosis

Carson, Sara; Psoter, Kevin J.; Koehler, Kirsten; Siklosi, Karen R.; Montemayor, Kristina; Toporek, A.; West, Natalie E.; Lechtzin, Noah; Hansel, Nadia N.; Collaco, Joseph M.; Merlo, Christian A.

doi:10.1016/j.jcf.2021.08.015

Cited by 8 publications

(1 citation statement)

References 29 publications

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“…Regarding environmental factors, the heterogeneity of phenotypes could be modulated by passive smoking [22,23], temperature [24], outdoor [25][26][27][28] and indoor air pollution [29], seasonality [30], climate and geography [31], socioeconomic status and access to health care [32,33]. The contribution of environmental factors is nevertheless variable across the phenotypes, ranging from 40% for variability in airway obstruction to 20% for nutritional status [5].…”

Section: Socio-demographic and Environmental Modifier Factorsmentioning

confidence: 99%

Modifier Factors of Cystic Fibrosis Phenotypes: A Focus on Modifier Genes

Mésinèle

Ruffin

Corvol

2022

IJMS

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Although cystic fibrosis (CF) is recognized as a monogenic disease, due to variants within the CFTR (Cystic Fibrosis Transmembrane Regulator) gene, an extreme clinical heterogeneity is described among people with CF (pwCF). Apart from the exocrine pancreatic status, most studies agree that there is little association between CFTR variants and disease phenotypes. Environmental factors have been shown to contribute to this heterogeneity, accounting for almost 50% of the variability of the lung function of pwCF. Nevertheless, pwCF with similar CFTR variants and sharing the same environment (such as in siblings) may have highly variable clinical manifestations not explained by CFTR variants, and only partly explained by environmental factors. It is recognized that genetic variants located outside the CFTR locus, named “modifier genes”, influence the clinical expression of the disease. This short review discusses the latest studies that have described modifier factors associated with the various CF phenotypes as well as the response to the recent CFTR modulator therapies.

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