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Clinical course and prognosis of never-smokers with severe alpha-1-antitrypsin deficiency (PiZZ).

Tanash, Hanan LU ; Nilsson, Peter M; Nilsson, Jan-Åke LU and Piitulainen, Eeva LU (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:
author
organization
publishing date
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
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
2008-09-11 16:26:05
date last changed
2016-10-30 04:36:29
@misc{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    = {ARRAY(0xa1aef60)},
  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},
  volume       = {63},
  year         = {2008},
}