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Intensive smoking diminishes the differences in quality of life and exacerbation frequency between the alpha-1-antitrypsin deficiency genotypes PiZZ and PiSZ.
- Source :
-
Respiratory medicine [Respir Med] 2017 Sep; Vol. 130, pp. 1-8. Date of Electronic Publication: 2017 Jul 08. - Publication Year :
- 2017
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Abstract
- Background: Alpha-1-antitrypsin deficiency (AATD) is a rare genetic disorder that is associated with low levels of circulating alpha-1-antitrypsin in serum. In comparison to the genotype PiZZ, PiSZ usually leads to lower risk of emphysema, better lung function and better survival. The aim of this study was to analyze the relationship between cigarette smoking (packyears) and the AATD genotypes (PiZZ and PiSZ) concerning quality of life (SGRQ), transfer factor of the lung for carbon monoxide (TLCO), forced expiratory volume in one second (FEV <subscript>1</subscript> ) and exacerbation rate.<br />Methods: We compared PiZZ and PiSZ individuals from the German registry for individuals with AATD (AATDR) in univariate analysis and multivariate linear regression models. All subjects were stratified into three groups according to their cumulative nicotine consumption (0 py; 0 < py < 30; ?30 py).<br />Results: 868 PiZZ individuals (mean age 52.6 ± 12.8 years (43.5% female)) and 114 PiSZ individuals (mean age 50.3 ± 17.4 years (46.5% female)) were compared. In contrast to never- and intensive (ex-) smokers, moderate (ex-) smoking PiSZ individuals had a significantly better SGRQ total score (B = ?8.148; p = 0.020) and less exacerbations (B = ?0.354; p = 0.037) than individuals with PiZZ in multivariate analysis.<br />Conclusions: The differences in quality of life and exacerbation frequency between PiZZ and PiSZ individuals diminish by intensive (ex-) smoking.<br /> (Copyright © 2017 Elsevier Ltd. All rights reserved.)
- Subjects :
- Adult
Aged
Carbon Monoxide metabolism
Disease Progression
Female
Forced Expiratory Volume physiology
Genotype
Germany epidemiology
Humans
Lung metabolism
Lung physiopathology
Lung Diseases complications
Lung Diseases epidemiology
Lung Diseases physiopathology
Male
Middle Aged
Quality of Life
Respiratory Function Tests methods
Smoking adverse effects
Smoking trends
alpha 1-Antitrypsin blood
Protease Inhibitors metabolism
Smoking physiopathology
Smoking psychology
alpha 1-Antitrypsin Deficiency genetics
alpha 1-Antitrypsin Deficiency physiopathology
alpha 1-Antitrypsin Deficiency psychology
Subjects
Details
- Language :
- English
- ISSN :
- 1532-3064
- Volume :
- 130
- Database :
- MEDLINE
- Journal :
- Respiratory medicine
- Publication Type :
- Academic Journal
- Accession number :
- 29206626
- Full Text :
- https://doi.org/10.1016/j.rmed.2017.07.004