PMID- 35023912
OWN - NLM
STAT- MEDLINE
DCOM- 20220322
LR  - 20220430
IS  - 1178-2005 (Electronic)
IS  - 1176-9106 (Print)
IS  - 1176-9106 (Linking)
VI  - 17
DP  - 2022
TI  - The Clinical Course of Severe Alpha-1-Antitrypsin Deficiency in Patients 
      Identified by Screening.
PG  - 43-52
LID - 10.2147/COPD.S340241 [doi]
AB  - BACKGROUND: Severe alpha-1-antitrypsin deficiency (AATD) is a genetic condition 
      predisposing to chronic obstructive pulmonary disease (COPD) and liver disease. 
      Its natural course is not well known. Our aim was to study the natural course of 
      AATD by analyzing the clinical course in individuals with severe AATD identified 
      by screening. MATERIALS AND METHODS: Of the 1585 individuals included in the 
      Swedish AATD register, 377 (24%) were identified by screening and included in 
      this retrospective study. The follow-up time was from the date of inclusion in 
      the register to the first lung transplantation, death or the termination of the 
      study on June 1st, 2016. The risk factors for having a diagnosis of COPD were 
      investigated through a proportional hazards model, adjusted for sex, diagnosis 
      before the age of 14 years, smoking habits, occupational exposure to airway 
      irritants and respiratory symptoms or diseases. RESULTS: At inclusion, 71% of the 
      individuals were asymptomatic, ie, without any respiratory symptoms. Compared to 
      the 156 (41%) ever-smokers, the 221 (59%) never-smokers had better lung function 
      (mean FEV(1) 98 (SD 18) vs 85 (SD 28) % predicted; p < 0.001), and fewer of them 
      were symptomatic, ie, with respiratory symptoms, at inclusion (20% vs 42%; p < 
      0.001). They also had a lower annual decline in FEV(1) (mean 42 (95% CI 36-47) vs 
      53 (95% CI 47-60) mL.yr(-1); p = 0.011) and better survival than the 
      ever-smokers. The risk factors for having a diagnosis of COPD were the 
      identification of severe AATD at an age of >/=14 years and the presence of 
      respiratory symptoms or diseases. CONCLUSION: Never-smoking individuals with 
      severe AATD identified by screening have better lung function, fewer symptoms, 
      and better survival compared with the ever-smokers. Screening for AATD at an 
      early age may improve the prognosis of AATD.
CI  - (c) 2022 Hiller et al.
FAU - Hiller, Adriana-Maria
AU  - Hiller AM
AUID- ORCID: 0000-0002-5452-0314
AD  - Department of Respiratory Medicine and Allergology, Lund University, Skane 
      University Hospital, Malmo, Sweden.
FAU - Piitulainen, Eeva
AU  - Piitulainen E
AD  - Department of Respiratory Medicine and Allergology, Lund University, Skane 
      University Hospital, Malmo, Sweden.
FAU - Tanash, Hanan
AU  - Tanash H
AD  - Department of Respiratory Medicine and Allergology, Lund University, Skane 
      University Hospital, Malmo, Sweden.
LA  - eng
PT  - Journal Article
DEP - 20220105
PL  - New Zealand
TA  - Int J Chron Obstruct Pulmon Dis
JT  - International journal of chronic obstructive pulmonary disease
JID - 101273481
SB  - IM
MH  - Adolescent
MH  - Humans
MH  - *Lung Transplantation/adverse effects
MH  - *Pulmonary Disease, Chronic Obstructive/diagnosis/etiology
MH  - Retrospective Studies
MH  - Smoking/adverse effects
MH  - *alpha 1-Antitrypsin Deficiency/complications/diagnosis/genetics
PMC - PMC8743380
OTO - NOTNLM
OT  - alpha-1-antitrypsin
OT  - clinical course
OT  - lung function
OT  - prognosis
OT  - screening
COIS- The authors report no conflicts of interest. The authors alone are responsible 
      for the content and writing of the paper.
EDAT- 2022/01/14 06:00
MHDA- 2022/03/23 06:00
PMCR- 2022/01/05
CRDT- 2022/01/13 12:25
PHST- 2021/09/20 00:00 [received]
PHST- 2021/12/06 00:00 [accepted]
PHST- 2022/01/13 12:25 [entrez]
PHST- 2022/01/14 06:00 [pubmed]
PHST- 2022/03/23 06:00 [medline]
PHST- 2022/01/05 00:00 [pmc-release]
AID - 340241 [pii]
AID - 10.2147/COPD.S340241 [doi]
PST - epublish
SO  - Int J Chron Obstruct Pulmon Dis. 2022 Jan 5;17:43-52. doi: 10.2147/COPD.S340241. 
      eCollection 2022.