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Survival in individuals with severe alpha 1-antitrypsin deficiency (PiZZ) in comparison to a general population with known smoking habits

Tanash, Hanan A. LU ; Ekström, Magnus LU ; Rönmark, Eva; Lindberg, Anne LU and Piitulainen, Eeva LU (2017) In European Respiratory Journal1988-01-01+01:00 50(3).
Abstract

Knowledge about the natural history of severe alpha 1-antitrypsin (AAT) deficiency (PiZZ) is limited. Our aim was to compare the survival of PiZZ individuals with randomly selected controls from the Swedish general population. The PiZZ subjects (n=1585) were selected from the Swedish National AATD Register. The controls (n=5999) were randomly selected from the Swedish population register. Smoking habits were known for all subjects. Median follow-up times for the PiZZ subjects (731 never-smokers) and controls (3179 never-smokers) were 12 and 17 years, respectively (p<0.001). During follow-up, 473 PiZZ subjects (30%), and 747 controls (12%) died. The PiZZ subjects had a significantly shorter survival time than the controls, p<0.001.... (More)

Knowledge about the natural history of severe alpha 1-antitrypsin (AAT) deficiency (PiZZ) is limited. Our aim was to compare the survival of PiZZ individuals with randomly selected controls from the Swedish general population. The PiZZ subjects (n=1585) were selected from the Swedish National AATD Register. The controls (n=5999) were randomly selected from the Swedish population register. Smoking habits were known for all subjects. Median follow-up times for the PiZZ subjects (731 never-smokers) and controls (3179 never-smokers) were 12 and 17 years, respectively (p<0.001). During follow-up, 473 PiZZ subjects (30%), and 747 controls (12%) died. The PiZZ subjects had a significantly shorter survival time than the controls, p<0.001. After adjustment for gender, age, smoking habits and presence of respiratory symptoms, the risk of death was still significantly higher for the PiZZ individuals than for the controls, hazard ratio (HR) 3.2 (95% CI 2.8-3.6; p<0.001). By contrast, the risk of death was not increased in never-smoking PiZZ individuals identified by screening, compared to never-smoking controls, HR 1.2 (95% CI 0.6-2.2). The never-smoking PiZZ individuals identified by screening had a similar life expectancy to the neversmokers in the Swedish general population. Early diagnosis of AAT deficiency is of utmost importance.

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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
alpha 1-antitrypsin, Smoking, Survival
in
European Respiratory Journal1988-01-01+01:00
volume
50
issue
3
publisher
Eur Respiratory Soc
external identifiers
  • scopus:85030033028
  • pmid:28889108
  • wos:000410089300008
ISSN
0903-1936
DOI
10.1183/13993003.00198-2017
language
English
LU publication?
yes
id
5c9cd09b-d392-4fef-82be-e7c610450a5f
date added to LUP
2017-10-09 13:49:14
date last changed
2018-11-21 21:35:06
@article{5c9cd09b-d392-4fef-82be-e7c610450a5f,
  abstract     = {<p>Knowledge about the natural history of severe alpha 1-antitrypsin (AAT) deficiency (PiZZ) is limited. Our aim was to compare the survival of PiZZ individuals with randomly selected controls from the Swedish general population. The PiZZ subjects (n=1585) were selected from the Swedish National AATD Register. The controls (n=5999) were randomly selected from the Swedish population register. Smoking habits were known for all subjects. Median follow-up times for the PiZZ subjects (731 never-smokers) and controls (3179 never-smokers) were 12 and 17 years, respectively (p&lt;0.001). During follow-up, 473 PiZZ subjects (30%), and 747 controls (12%) died. The PiZZ subjects had a significantly shorter survival time than the controls, p&lt;0.001. After adjustment for gender, age, smoking habits and presence of respiratory symptoms, the risk of death was still significantly higher for the PiZZ individuals than for the controls, hazard ratio (HR) 3.2 (95% CI 2.8-3.6; p&lt;0.001). By contrast, the risk of death was not increased in never-smoking PiZZ individuals identified by screening, compared to never-smoking controls, HR 1.2 (95% CI 0.6-2.2). The never-smoking PiZZ individuals identified by screening had a similar life expectancy to the neversmokers in the Swedish general population. Early diagnosis of AAT deficiency is of utmost importance.</p>},
  articleno    = {00198},
  author       = {Tanash, Hanan A. and Ekström, Magnus and Rönmark, Eva and Lindberg, Anne and Piitulainen, Eeva},
  issn         = {0903-1936},
  keyword      = {alpha 1-antitrypsin,Smoking,Survival},
  language     = {eng},
  month        = {09},
  number       = {3},
  publisher    = {Eur Respiratory Soc},
  series       = {European Respiratory Journal1988-01-01+01:00},
  title        = {Survival in individuals with severe alpha 1-antitrypsin deficiency (PiZZ) in comparison to a general population with known smoking habits},
  url          = {http://dx.doi.org/10.1183/13993003.00198-2017},
  volume       = {50},
  year         = {2017},
}