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Survival in severe alpha-1-antitrypsin deficiency (PiZZ)

Tanash, Hanan LU ; Nilsson, Peter LU ; Nilsson, Jan-Åke LU and Piitulainen, Eeva LU (2010) In Respiratory Research 11.
Abstract
Background: Previous studies of the natural history of alpha-1-antitrypsin (AAT) deficiency are mostly based on highly selected patients. The aim of this study was to analyse the mortality of PiZZ individuals. Methods: Data from 1339 adult PiZZ individuals from the Swedish National AAT Deficiency Registry, followed from 1991 to 2008, were analysed. Forty-three percent of these individuals were identified by respiratory symptoms (respiratory cases), 32% by liver diseases and other diseases (non-respiratory cases) and 25% by screening (screened cases). Smoking status was divided into two groups: smokers 737 (55%) and 602 (45%) never-smokers. Results: During the follow-up 315 individuals (24%) died. The standardised mortality rate (SMR) for... (More)
Background: Previous studies of the natural history of alpha-1-antitrypsin (AAT) deficiency are mostly based on highly selected patients. The aim of this study was to analyse the mortality of PiZZ individuals. Methods: Data from 1339 adult PiZZ individuals from the Swedish National AAT Deficiency Registry, followed from 1991 to 2008, were analysed. Forty-three percent of these individuals were identified by respiratory symptoms (respiratory cases), 32% by liver diseases and other diseases (non-respiratory cases) and 25% by screening (screened cases). Smoking status was divided into two groups: smokers 737 (55%) and 602 (45%) never-smokers. Results: During the follow-up 315 individuals (24%) died. The standardised mortality rate (SMR) for respiratory cases was 4.70 (95% Confidence Interval (CI) 4.10-5.40), 3.0 (95% CI 2.35-3.70) for the non-respiratory cases and 2.30 (95% CI 1.46-3.46) for the screened cases. The smokers had a higher mortality risk than never-smokers, with a SMR of 4.80 (95% CI 4.20-5.50) for the smokers and 2.80(95% CI 2.30-3.40) for the never-smokers. The Rate Ratio (RR) was 1.70 (95% CI 1.35-2.20). Also among the screened cases, the mortality risk for the smokers was significantly higher than in the general Swedish population (SMR 3.40 (95% CI 1.98-5.40). Conclusion: Smokers with severe AAT deficiency, irrespective of mode of identification, have a significantly higher mortality risk than the general Swedish population. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Respiratory Research
volume
11
publisher
BioMed Central
external identifiers
  • wos:000278327800001
  • scopus:77954639932
ISSN
1465-9921
DOI
10.1186/1465-9921-11-44
language
English
LU publication?
yes
id
aec0062e-01b4-4cf3-913d-90caeaa2ac73 (old id 1631646)
date added to LUP
2010-07-21 11:15:23
date last changed
2018-05-29 12:24:08
@article{aec0062e-01b4-4cf3-913d-90caeaa2ac73,
  abstract     = {Background: Previous studies of the natural history of alpha-1-antitrypsin (AAT) deficiency are mostly based on highly selected patients. The aim of this study was to analyse the mortality of PiZZ individuals. Methods: Data from 1339 adult PiZZ individuals from the Swedish National AAT Deficiency Registry, followed from 1991 to 2008, were analysed. Forty-three percent of these individuals were identified by respiratory symptoms (respiratory cases), 32% by liver diseases and other diseases (non-respiratory cases) and 25% by screening (screened cases). Smoking status was divided into two groups: smokers 737 (55%) and 602 (45%) never-smokers. Results: During the follow-up 315 individuals (24%) died. The standardised mortality rate (SMR) for respiratory cases was 4.70 (95% Confidence Interval (CI) 4.10-5.40), 3.0 (95% CI 2.35-3.70) for the non-respiratory cases and 2.30 (95% CI 1.46-3.46) for the screened cases. The smokers had a higher mortality risk than never-smokers, with a SMR of 4.80 (95% CI 4.20-5.50) for the smokers and 2.80(95% CI 2.30-3.40) for the never-smokers. The Rate Ratio (RR) was 1.70 (95% CI 1.35-2.20). Also among the screened cases, the mortality risk for the smokers was significantly higher than in the general Swedish population (SMR 3.40 (95% CI 1.98-5.40). Conclusion: Smokers with severe AAT deficiency, irrespective of mode of identification, have a significantly higher mortality risk than the general Swedish population.},
  author       = {Tanash, Hanan and Nilsson, Peter and Nilsson, Jan-Åke and Piitulainen, Eeva},
  issn         = {1465-9921},
  language     = {eng},
  publisher    = {BioMed Central},
  series       = {Respiratory Research},
  title        = {Survival in severe alpha-1-antitrypsin deficiency (PiZZ)},
  url          = {http://dx.doi.org/10.1186/1465-9921-11-44},
  volume       = {11},
  year         = {2010},
}