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Survival in individuals with severe alpha 1-antitrypsin deficiency (PiZZ) in comparison to a general population with known smoking habits
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Occupational and Environmental Medicine.
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
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2017 (English)In: European Respiratory Journal, ISSN 0903-1936, E-ISSN 1399-3003, Vol. 50, no 3, 1700198Article in journal (Refereed) Published
Abstract [en]

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 2.83.6; 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.

Place, publisher, year, edition, pages
EUROPEAN RESPIRATORY SOC JOURNALS LTD , 2017. Vol. 50, no 3, 1700198
National Category
Respiratory Medicine and Allergy
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URN: urn:nbn:se:umu:diva-140046DOI: 10.1183/13993003.00198-2017ISI: 000410089300008PubMedID: 28889108OAI: oai:DiVA.org:umu-140046DiVA: diva2:1146129
Available from: 2017-10-02 Created: 2017-10-02 Last updated: 2017-10-16Bibliographically approved

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CiteExportLink to record
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Citation style
  • apa
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