title: Lung function in 30-year-old alpha-1-antitrypsin-deficient individuals.
abstract: BACKGROUND
  Alpha-1-antitrypsin (AAT) deficiency increases the risk of emphysema, especially in smokers. In 1972-1974, all 200,000 Swedish new-born infants were screened for AAT deficiency and individuals with severe (PiZZ) and moderate (PiSZ) deficiency have been followed-up regularly. The aim of the present study was to examine their lung function at the age of 30 years, comparing them to a group of age-matched control subjects (PiMM) recruited from the general population, and to compare current smokers with never-smokers.
  
  
  METHODS
  Static and dynamic spirometry, including TLC, FRC, RV, VC, FEV(1,)K(CO) and D(L,CO), was performed for all participants. All values were expressed as percentages of the expected values. FEV(1)/VC was expressed both as percentage of the expected value and in absolute numbers.
  
  
  RESULTS
  Four of 60 PiZZ, none of 19 PiSZ and 9 of 33 PiMM participating individuals were current smokers. All Pi groups had a normal mean FEV(1). The mean (SD) FEV(1)/VC ratio was 75% (7.4) in the PiZZ smokers and 84% (5.5) in the PiZZ never-smokers (p<0.01). The mean (SD) K(CO) was 81 (13) in the PiZZ smokers and 99 (14) in the PiZZ never-smokers (p<0.05).
  
  
  CONCLUSIONS
  AAT-deficient individuals identified by neonatal screening have normal lung function at the age of 30. The PiZZ smokers had changes in lung function that may be signs of early emphysema.
authors.affiliation: Department of Respiratory Medicine, Lund University, University Hospital, Entrance 35, SE-205 02 Malmö, Sweden. elisabeth.bernspang@med.lu.se
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Bernspång
authors.forename: Per
authors.initials: P
authors.lastname: Wollmer
authors.forename: Tomas
authors.initials: T
authors.lastname: Sveger
authors.forename: Eeva
authors.initials: E
authors.lastname: Piitulainen
mesh_terms: D000328:Adult; D000367:Age Factors; D016022:Case-Control Studies; D015331:Cohort Studies; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000503:physiopathology*; D008297:Male; D015997:Neonatal Screening; D010641:Phenotype; D012907:Smoking / Q000009:adverse effects; D013147:Spirometry; D013548:Sweden / Q000453:epidemiology; D014797:Vital Capacity; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications / Q000503:physiopathology*