Survival in severe alpha-1-antitrypsin deficiency (PiZZ)
(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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https://lup.lub.lu.se/record/1631646
- author
- Tanash, Hanan LU ; Nilsson, Peter LU ; Nilsson, Jan-Åke LU and Piitulainen, Eeva LU
- organization
- publishing date
- 2010
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Respiratory Research
- volume
- 11
- publisher
- BioMed Central (BMC)
- external identifiers
-
- wos:000278327800001
- scopus:77954639932
- pmid:20420704
- 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
- 2016-04-01 10:19:18
- date last changed
- 2024-04-21 09:24:01
@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 (BMC)}},
series = {{Respiratory Research}},
title = {{Survival in severe alpha-1-antitrypsin deficiency (PiZZ)}},
url = {{http://dx.doi.org/10.1186/1465-9921-11-44}},
doi = {{10.1186/1465-9921-11-44}},
volume = {{11}},
year = {{2010}},
}