LUNG-FUNCTION IN ADOLESCENTS WITH ALPHA(1)-ANTITRYPSIN DEFICIENCY

Children with alpha(1)-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 questi onnaire and asked to participate in an extensive lung function study i ncluding part or all of the following tests: FVC, FEV(1) before and 15 min after four inhaled doses of salbutamol, TLC, RV and FRC. Fifty ag e-, sex- and height-matched adolescents participated as controls. No s ignificant differences in age, height or weight were found between the PiZ, PiSZ and control groups. No significant differences were found i n 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 Pi Z and 1 of 45 PiSZ adolescents were smokers. No significant contributi on of al-antitrypsin Pi-type was found to explain the variation in lun g function variables studied. We conclude that children with alpha(1)- antitrypsin deficiency have a favourable prognosis and normal lung dev elopment up to 16 years of age. Anti-smoking advice was found to be re asonably successful; only 3% of those answering the questionnaire had started to smoke.