Why is Disease Penetration so Variable in Alpha-1 Antitrypsin Deficiency? The Contribution of Environmental Factors

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@article{a77f8fb7c59144b7bf4340f8fe521099,
title = "Why is Disease Penetration so Variable in Alpha-1 Antitrypsin Deficiency? The Contribution of Environmental Factors",
abstract = "Environmental influences on clinical phenotype in alpha-1 antitrypsin deficiency (AATD) include cigarette smoke, occupational exposures, airway/sputum bacteria and outdoor air pollution. This narrative review describes the impact of the major environmental exposures and summarizes their effect on clinical phenotype and outcomes. In general, patients with AATD are more susceptible to pulmonary damage as a result of the relatively unopposed action of neutrophil elastase, in the context of neutrophilic inflammation stimulated by environmental factors. However, the amount of phenotypic variability explicable by environmental factors is insufficient to account for the wide range of clinical presentations observed, suggesting that a combination of genetic and environmental factors is likely to be responsible. ",
keywords = "Alpha 1 antitrypsin deficiency, microbiota, occupational exposure, cigarette smoking, air pollution",
author = "Madhu Rangaraju and Alice Turner",
year = "2020",
month = jul,
day = "31",
doi = "10.15326/jcopdf.7.3.2019.0177",
language = "English",
pages = "280--289",
journal = "Chronic Obstructive Pulmonary Diseases: Journal of the COPD Foundation",
issn = "2372-952X",
publisher = "COPD Foundation",

}

RIS

TY - JOUR

T1 - Why is Disease Penetration so Variable in Alpha-1 Antitrypsin Deficiency? The Contribution of Environmental Factors

AU - Rangaraju, Madhu

AU - Turner, Alice

PY - 2020/7/31

Y1 - 2020/7/31

N2 - Environmental influences on clinical phenotype in alpha-1 antitrypsin deficiency (AATD) include cigarette smoke, occupational exposures, airway/sputum bacteria and outdoor air pollution. This narrative review describes the impact of the major environmental exposures and summarizes their effect on clinical phenotype and outcomes. In general, patients with AATD are more susceptible to pulmonary damage as a result of the relatively unopposed action of neutrophil elastase, in the context of neutrophilic inflammation stimulated by environmental factors. However, the amount of phenotypic variability explicable by environmental factors is insufficient to account for the wide range of clinical presentations observed, suggesting that a combination of genetic and environmental factors is likely to be responsible.

AB - Environmental influences on clinical phenotype in alpha-1 antitrypsin deficiency (AATD) include cigarette smoke, occupational exposures, airway/sputum bacteria and outdoor air pollution. This narrative review describes the impact of the major environmental exposures and summarizes their effect on clinical phenotype and outcomes. In general, patients with AATD are more susceptible to pulmonary damage as a result of the relatively unopposed action of neutrophil elastase, in the context of neutrophilic inflammation stimulated by environmental factors. However, the amount of phenotypic variability explicable by environmental factors is insufficient to account for the wide range of clinical presentations observed, suggesting that a combination of genetic and environmental factors is likely to be responsible.

KW - Alpha 1 antitrypsin deficiency

KW - microbiota

KW - occupational exposure

KW - cigarette smoking

KW - air pollution

UR - https://journal.copdfoundation.org/

UR - https://journal.copdfoundation.org/About-Us

UR - https://journal.copdfoundation.org/Documents/JCOPDF-Instructions-for-Authors.pdf

U2 - 10.15326/jcopdf.7.3.2019.0177

DO - 10.15326/jcopdf.7.3.2019.0177

M3 - Review article

SP - 280

EP - 289

JO - Chronic Obstructive Pulmonary Diseases: Journal of the COPD Foundation

JF - Chronic Obstructive Pulmonary Diseases: Journal of the COPD Foundation

SN - 2372-952X

ER -