Clinical course and prognosis of never-smokers with severe alpha-1-antitrypsin deficiency (PiZZ).
(2008) In Thorax 63. p.1091-1095- Abstract
- BACKGROUND: Previous studies of non-smoking individuals with severe alpha-1-antitrypsin deficiency (PiZZ) have been sparse and included only a limited number of individuals, mostly identified by respiratory symptoms. The aim of this study was to estimate the prognosis of non-smoking PiZZ individuals and to analyze the most common causes of death by including a large number of individuals who had been identified due to reasons other than respiratory symptoms. METHODS: The study included 568 non-smoking PiZZ subjects who were selected from the Swedish National AAT Deficiency Registry and followed-up from 1991 to September 2007. Of these, 156 (27%) were identified by respiratory symptoms (respiratory cases) and 412 were identified by extra... (More)
- BACKGROUND: Previous studies of non-smoking individuals with severe alpha-1-antitrypsin deficiency (PiZZ) have been sparse and included only a limited number of individuals, mostly identified by respiratory symptoms. The aim of this study was to estimate the prognosis of non-smoking PiZZ individuals and to analyze the most common causes of death by including a large number of individuals who had been identified due to reasons other than respiratory symptoms. METHODS: The study included 568 non-smoking PiZZ subjects who were selected from the Swedish National AAT Deficiency Registry and followed-up from 1991 to September 2007. Of these, 156 (27%) were identified by respiratory symptoms (respiratory cases) and 412 were identified by extra pulmonary symptoms or screening (non-respiratory cases). RESULTS: Ninety-three subjects (16%) died during the follow-up time. The specific Standardized Mortality Rate (SMR) for the whole study population was 2.32 (95% confidence interval (CI) 1.87-2.83) with no significant difference between men and women. The SMR was 2.55 (95% CI 1.91-2.83) for the respiratory cases and 2.07 (95% CI 1.49-2.81) for the non-respiratory cases. Further calculation of SMR for subgroups in the non-respiratory cases showed that the SMR was 0.70 (95% CI 0.14-2.04) for individuals identified by family/population screening. Emphysema and liver cirrhosis were the most common causes of death (45% and 28%, respectively). Malignant transformation was found in 38% of the cirrhotic cases. CONCLUSION: Non-smoking PiZZ individuals identified by screening do not have an increased mortality risk compared with the Swedish general population. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/1223450
- author
- Tanash, Hanan LU ; Nilsson, Peter M LU ; Nilsson, Jan-Åke LU and Piitulainen, Eeva LU
- organization
- publishing date
- 2008
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Thorax
- volume
- 63
- pages
- 1091 - 1095
- publisher
- BMJ Publishing Group
- external identifiers
-
- wos:000261033800014
- pmid:18682522
- scopus:57149107426
- pmid:18682522
- ISSN
- 1468-3296
- DOI
- 10.1136/thx.2008.095497
- language
- English
- LU publication?
- yes
- id
- bb4f261b-b2ec-4bf8-a867-7617836add1b (old id 1223450)
- alternative location
- http://www.ncbi.nlm.nih.gov/pubmed/18682522?dopt=Abstract
- date added to LUP
- 2016-04-04 09:34:14
- date last changed
- 2024-04-13 08:14:00
@article{bb4f261b-b2ec-4bf8-a867-7617836add1b,
abstract = {{BACKGROUND: Previous studies of non-smoking individuals with severe alpha-1-antitrypsin deficiency (PiZZ) have been sparse and included only a limited number of individuals, mostly identified by respiratory symptoms. The aim of this study was to estimate the prognosis of non-smoking PiZZ individuals and to analyze the most common causes of death by including a large number of individuals who had been identified due to reasons other than respiratory symptoms. METHODS: The study included 568 non-smoking PiZZ subjects who were selected from the Swedish National AAT Deficiency Registry and followed-up from 1991 to September 2007. Of these, 156 (27%) were identified by respiratory symptoms (respiratory cases) and 412 were identified by extra pulmonary symptoms or screening (non-respiratory cases). RESULTS: Ninety-three subjects (16%) died during the follow-up time. The specific Standardized Mortality Rate (SMR) for the whole study population was 2.32 (95% confidence interval (CI) 1.87-2.83) with no significant difference between men and women. The SMR was 2.55 (95% CI 1.91-2.83) for the respiratory cases and 2.07 (95% CI 1.49-2.81) for the non-respiratory cases. Further calculation of SMR for subgroups in the non-respiratory cases showed that the SMR was 0.70 (95% CI 0.14-2.04) for individuals identified by family/population screening. Emphysema and liver cirrhosis were the most common causes of death (45% and 28%, respectively). Malignant transformation was found in 38% of the cirrhotic cases. CONCLUSION: Non-smoking PiZZ individuals identified by screening do not have an increased mortality risk compared with the Swedish general population.}},
author = {{Tanash, Hanan and Nilsson, Peter M and Nilsson, Jan-Åke and Piitulainen, Eeva}},
issn = {{1468-3296}},
language = {{eng}},
pages = {{1091--1095}},
publisher = {{BMJ Publishing Group}},
series = {{Thorax}},
title = {{Clinical course and prognosis of never-smokers with severe alpha-1-antitrypsin deficiency (PiZZ).}},
url = {{http://dx.doi.org/10.1136/thx.2008.095497}},
doi = {{10.1136/thx.2008.095497}},
volume = {{63}},
year = {{2008}},
}