Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học 'Respiratory Research cung cấp cho các bạn kiến thức về ngành y đề tài:"Survival in severe alpha-1-antitrypsin deficiency (PiZZ). | Tanash et al. Respiratory Research 2010 11 44 http content 11 1 44 RESPIRATORY RESEARCH RESEARCH Open Access Survival in severe alpha-1-antitrypsin deficiency PiZZ Hanan ATanash 1 PeterM Nilsson2 Jan-Ake Nilsson1 and Eeva Piitulainen1 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 95 Confidence Interval CI 95 CI for the non-respiratory cases and 95 CI for the screened cases. The smokers had a higher mortality risk than never-smokers with a SMR of 95 CI for the smokers and 95 CI for the never-smokers. The Rate Ratio RR was 95 CI . Also among the screened cases the mortality risk for the smokers was significantly higher than in the general Swedish population SMR 95 CI . Conclusion Smokers with severe AAT deficiency irrespective of mode of identification have a significantly higher mortality risk than the general Swedish population. Introduction Severe alpha-1-antitrypsin deficiency AATD is a hereditary condition characterised by low levels of AAT in serum and the lungs a high risk of developing panacinar emphysema and an increased risk of liver disease primarily in early childhood and late adulthood 1-6 . Cigarette smoking is strongly associated .
