title: Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ.
abstract: Lung function, smoking, age and mortality data in 158 adult severe alpha 1-antitrypsin deficient, PiZZ individuals, followed from 1963 to 1982 were analyzed. Low initial FEV1 value was significantly associated with increased mortality (p less than 0.005). A 3 yr mortality rate of 40% was found in individuals whose initial FEV1 values were less than 30% of that predicted. In contrast, the corresponding 3-yr mortality among those whose initial FEV1 values were between 30 and 65% of that predicted was only 7%. Smokers were found to have significantly lower FEV1 levels (p = 0.008) and higher mortality (p less than 0.005) than non-smokers. The difference between current and ex-smokers in mortality and FEV1 level were not statistically significant (p = 0.9 and p greater than 0.25, respectively). Cross-sectional analysis of the initial FEV1 values indicated a significant decline (p less than 0.005) of FEV1 with increasing age. This decline was greater among smokers than non-smokers. Longitudinal analysis of FEV1 rates of decline in 80 cases with follow-up FEV1 measurements failed to detect any significant differences between smokers and non-smokers, but was performed late in the disease process. The application of these results to the planning of studies on replacement therapy, smoking intervention strategy and longitudinal follow-up is discussed.
authors.affiliation: National Heart, Lung and Blood Institute, Bethesda, MD 20892.
authors.forename: M C
authors.initials: MC
authors.lastname: Wu
authors.forename: S
authors.initials: S
authors.lastname: Eriksson
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D001796:Blood Protein Disorders / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012189:Retrospective Studies; D012907:Smoking / Q000401:mortality / Q000503:physiopathology*; D013147:Spirometry; D013548:Sweden; D019896:alpha 1-Antitrypsin Deficiency