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Lung function and CT lung densitometry in 37-to 39-year-old individuals with alpha-1-antitrypsin deficiency

Mostafavi, Behrouz LU ; Diaz, Sandra LU ; Piitulainen, Eeva LU ; Stoel, Berend C. ; Wollmer, Per LU and Tanash, Hanan A. LU (2018) In International Journal of COPD 13. p.3689-3698
Abstract

Background: Alpha-1-antitrypsin (AAT) deficiency is a hereditary disorder that predisposes to emphysema. A cohort of severe (PiZZ) and moderate (PiSZ) AAT-deficient newborn infants was identified by the Swedish national neonatal AAT screening program in 1972–1974 and has been followed-up since birth. Our aim was to study whether the cohort has signs of emphysema in pulmonary function tests (PFTs) and computed tomography (CT) densitometry at 38 years of age in comparison with an age-matched control group, randomly selected from the population registry. Methods: Forty-one PiZZ, 18 PiSZ, and 61 control subjects (PiMM) underwent complete PFTs, measurement of resistance and reactance in the respiratory system by impulse oscillometry... (More)

Background: Alpha-1-antitrypsin (AAT) deficiency is a hereditary disorder that predisposes to emphysema. A cohort of severe (PiZZ) and moderate (PiSZ) AAT-deficient newborn infants was identified by the Swedish national neonatal AAT screening program in 1972–1974 and has been followed-up since birth. Our aim was to study whether the cohort has signs of emphysema in pulmonary function tests (PFTs) and computed tomography (CT) densitometry at 38 years of age in comparison with an age-matched control group, randomly selected from the population registry. Methods: Forty-one PiZZ, 18 PiSZ, and 61 control subjects (PiMM) underwent complete PFTs, measurement of resistance and reactance in the respiratory system by impulse oscillometry (IOS)/forced oscillation technique (FOT), and CT densitometry. The results were related to self-reported smoking habits. Results: The total lung capacity (TLC) % of the predicted value was significantly higher in the PiZZ ever-smokers than in the PiZZ never-smokers (P<0.05), PiSZ never-smokers (P=0.01) and the PiMM never-smokers (P=0.01). The residual volume (RV) % of the predicted value was significantly higher in the PiZZ ever-smokers compared to the PiMM never-smokers (P<0.01). The PiZZ ever-smokers had a significantly lower carbon monoxide transfer coefficient (Kco) than the PiSZ never-smokers (P<0.01) and PiMM never-smokers (P<0.01). Respiratory system resistance at 5 Hz (P<0.01), at 20 Hz (P<0.01), and the area of low reactance (Alx; P<0.05) were significantly lower and respiratory system reactance at 5 Hz (P<0.05) was significantly higher in PiZZ subjects compared to the PiMM subjects. No statistically significant differences in the CT densitometry parameters were found between the Pi subgroups. Conclusion: The physiological parameters in the PiZZ ever-smokers showed evidence of hyperinflation and emphysema before the age of 40 years.

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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Alpha-1-antitrypsin deficiency, Impulse oscillometry, Lung disease, Lung function, Screening
in
International Journal of COPD
volume
13
pages
10 pages
publisher
Dove Press
external identifiers
  • pmid:30510411
  • scopus:85058496468
ISSN
1176-9106
DOI
10.2147/COPD.S167497
language
English
LU publication?
yes
id
3ca35b84-aa5e-4b9c-a8cb-a2875268cad3
date added to LUP
2019-01-07 13:25:06
date last changed
2020-07-08 04:37:44
@article{3ca35b84-aa5e-4b9c-a8cb-a2875268cad3,
  abstract     = {<p>Background: Alpha-1-antitrypsin (AAT) deficiency is a hereditary disorder that predisposes to emphysema. A cohort of severe (PiZZ) and moderate (PiSZ) AAT-deficient newborn infants was identified by the Swedish national neonatal AAT screening program in 1972–1974 and has been followed-up since birth. Our aim was to study whether the cohort has signs of emphysema in pulmonary function tests (PFTs) and computed tomography (CT) densitometry at 38 years of age in comparison with an age-matched control group, randomly selected from the population registry. Methods: Forty-one PiZZ, 18 PiSZ, and 61 control subjects (PiMM) underwent complete PFTs, measurement of resistance and reactance in the respiratory system by impulse oscillometry (IOS)/forced oscillation technique (FOT), and CT densitometry. The results were related to self-reported smoking habits. Results: The total lung capacity (TLC) % of the predicted value was significantly higher in the PiZZ ever-smokers than in the PiZZ never-smokers (P&lt;0.05), PiSZ never-smokers (P=0.01) and the PiMM never-smokers (P=0.01). The residual volume (RV) % of the predicted value was significantly higher in the PiZZ ever-smokers compared to the PiMM never-smokers (P&lt;0.01). The PiZZ ever-smokers had a significantly lower carbon monoxide transfer coefficient (Kco) than the PiSZ never-smokers (P&lt;0.01) and PiMM never-smokers (P&lt;0.01). Respiratory system resistance at 5 Hz (P&lt;0.01), at 20 Hz (P&lt;0.01), and the area of low reactance (Alx; P&lt;0.05) were significantly lower and respiratory system reactance at 5 Hz (P&lt;0.05) was significantly higher in PiZZ subjects compared to the PiMM subjects. No statistically significant differences in the CT densitometry parameters were found between the Pi subgroups. Conclusion: The physiological parameters in the PiZZ ever-smokers showed evidence of hyperinflation and emphysema before the age of 40 years.</p>},
  author       = {Mostafavi, Behrouz and Diaz, Sandra and Piitulainen, Eeva and Stoel, Berend C. and Wollmer, Per and Tanash, Hanan A.},
  issn         = {1176-9106},
  language     = {eng},
  pages        = {3689--3698},
  publisher    = {Dove Press},
  series       = {International Journal of COPD},
  title        = {Lung function and CT lung densitometry in 37-to 39-year-old individuals with alpha-1-antitrypsin deficiency},
  url          = {http://dx.doi.org/10.2147/COPD.S167497},
  doi          = {10.2147/COPD.S167497},
  volume       = {13},
  year         = {2018},
}