Lung function in adolescents with α1-antitrypsin deficiency

Children with α₁-antitrypsin deficiency, screened at birth, were followed prospectively. At 16 years of age, 150 adolescents (103 PiZ, 1 PiZ-, 1 PiS-, 45 PiSZ) were interviewed using a standardized questionnaire and asked to participate in an extensive lung function study including part or all of the following tests: FVC, FEV₁ before and 15 min after four inhaled doses of salbutamol, TLC, RV and FRC. Fifty age-, sex- and height-matched adolescents participated as controls. No significant differences in age, height or weight were found between the PiZ, PiSZ and control groups. No significant differences were found in respiratory symptoms, parental smoking history or the smoking habits of PiZ, PiSZ and control subjects. Asthma occurred in 10.7% of PiZ, 6.5% of PiSZ and 4% of control adolescents (p = 0.33). Only 3 of 100 PiZ and 1 of 45 PiSZ adolescents were smokers. No significant contribution of α₁-antitrypsin Pi-type was found to explain the variation in lung function variables studied. We conclude that children with α₁-antitrypsin deficiency have a favourable prognosis and normal lung development up to 16 years of age. Anti-smoking advice was found to be reasonably successful; only 3% of those answering the questionnaire had started to smoke.