PMID- 34992356
OWN - NLM
STAT- MEDLINE
DCOM- 20220322
LR  - 20220429
IS  - 1178-2005 (Electronic)
IS  - 1176-9106 (Print)
IS  - 1176-9106 (Linking)
VI  - 16
DP  - 2021
TI  - Lung Function and Health Status in Individuals with Severe Alpha-1-Antitrypsin 
      Deficiency at the Age of 42.
PG  - 3477-3485
LID - 10.2147/COPD.S335683 [doi]
AB  - BACKGROUND: Severe hereditary alpha-1-antitrypsin deficiency (AATD) is a known 
      risk factor for the early development of pulmonary emphysema and COPD, especially 
      in smokers. By the Swedish national screening programme carried out from 1972 to 
      1974, a cohort of individuals with severe (PiZZ) AATD was identified and has been 
      followed up regularly. The aim of this study was to investigate health status, 
      quality of life and lung function in this cohort at the age of 42 years compared 
      with an age-matched control group randomly selected from the population registry. 
      METHODS: All study participants answered a questionnaire on smoking habits, 
      symptoms, occupation, exposure to airway irritants and quality of life using 
      Saint George's Respiratory Questionnaire (SGRQ). They underwent complete 
      pulmonary function tests (PFT) and forced oscillation technique (FOT) for the 
      measurement of airway resistance and reactance. Blood samples were taken for 
      allergies and IgG-subclasses as an indicator of increased risk of airway 
      infections. RESULTS: The residual volume (RV), total lung capacity (TLC) and 
      RV/TLC ratio were significantly higher in the PiZZ ever-smokers compared to the 
      PiMM ever-smokers and PiZZ never-smokers (p < 0.05). The resistance in the upper, 
      small and total airways was significantly lower in PiZZ subjects compared to PiMM 
      subjects (p < 0.05). A greater proportion of PiZZ never-smokers had an FEV(1)/VC 
      ratio <0.7 than PiMM never-smokers (p = 0.043). PiZZ subjects with occupational 
      exposure to airway irritants showed a significantly lower FEV(1), VC and higher 
      RV/TLC ratio than PiMM individuals with exposure (p < 0.05). CONCLUSION: At the 
      age of 42, ever-smoking PiZZ individuals have signs of COPD, and also PiZZ 
      never-smokers have early, physiological signs of emphysema.
CI  - (c) 2021 Schramm et al.
FAU - Schramm, Georg Rudiger
AU  - Schramm GR
AUID- ORCID: 0000-0002-8069-1539
AD  - Department of Respiratory Medicine and Allergology, Skane University Hospital, 
      Lund University, Malmo, Sweden.
FAU - Mostafavi, Behrouz
AU  - Mostafavi B
AD  - Department of Respiratory Medicine and Allergology, Skane University Hospital, 
      Lund University, Malmo, Sweden.
FAU - Piitulainen, Eeva
AU  - Piitulainen E
AD  - Department of Respiratory Medicine and Allergology, Skane University Hospital, 
      Lund University, Malmo, Sweden.
FAU - Wollmer, Per
AU  - Wollmer P
AD  - Department of Medical Imaging and Physiology, Skane University Hospital, Lund 
      University, Malmo, Sweden.
FAU - Tanash, Hanan A
AU  - Tanash HA
AD  - Department of Respiratory Medicine and Allergology, Skane University Hospital, 
      Lund University, Malmo, Sweden.
LA  - eng
PT  - Journal Article
DEP - 20211223
PL  - New Zealand
TA  - Int J Chron Obstruct Pulmon Dis
JT  - International journal of chronic obstructive pulmonary disease
JID - 101273481
SB  - IM
MH  - Adult
MH  - Health Status
MH  - Humans
MH  - Lung
MH  - *Pulmonary Disease, Chronic Obstructive/diagnosis/epidemiology/etiology
MH  - Quality of Life
MH  - *alpha 1-Antitrypsin Deficiency/complications/diagnosis/epidemiology
PMC - PMC8711559
OTO - NOTNLM
OT  - COPD
OT  - alpha-1-antitrypsin deficiency
OT  - lung function
OT  - quality of life
COIS- Professor Per Wollmer reports grants from Swedish Heart and Lung Foundation, 
      during the conduct of the study; personal fees from Chiesi Pharma, outside the 
      submitted work. The authors report no other conflicts of interest. The authors 
      alone are responsible for the drafting as well as the content of this study.
EDAT- 2022/01/08 06:00
MHDA- 2022/03/23 06:00
PMCR- 2021/12/23
CRDT- 2022/01/07 06:19
PHST- 2021/09/04 00:00 [received]
PHST- 2021/11/14 00:00 [accepted]
PHST- 2022/01/07 06:19 [entrez]
PHST- 2022/01/08 06:00 [pubmed]
PHST- 2022/03/23 06:00 [medline]
PHST- 2021/12/23 00:00 [pmc-release]
AID - 335683 [pii]
AID - 10.2147/COPD.S335683 [doi]
PST - epublish
SO  - Int J Chron Obstruct Pulmon Dis. 2021 Dec 23;16:3477-3485. doi: 
      10.2147/COPD.S335683. eCollection 2021.