Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin

C. E. Vreim, M. Wu, R. G. Crystal, A. S. Buist, B. Burrows, A. B. Cohen, R. J. Fallat, J. E. Gadek, R. H. Rousell, R. S. Schwartz, G. M. Turino, M. D. Schluchter, J. K. Stoller, H. P. Wiedemann, G. W. Williams, D. M. Barrett, G. J. Beck, V. Midcalf, B. Moore, P. SartoriS. G. Sherer, R. Zhang, T. L. Petty, Jr Tomashefski, M. L. Brantly, J. Hildesheim, B. Rundquist, R. A. Sandhaus, C. W. Bell, J. Berend, B. Begle, D. Erb, J. Seidman, S. Sherman, B. Cameron, S. Weinberger, M. Rosenberg, R. Johnston, M. Rosenberg, A. C. Arroliga, D. P. Meeker, A. Mehta, D. Laskowski, J. Ryan, J. P. Loftin, K. Johnson, A. Kotch, T. Clark, P. E. Epstein, P. Del Buono, R. E. Sandblom, R. C. Hert, J. B. DeMaine, L. Colar, M. S. Eichenhorn, J. P. McMahan, W. M. Breite, D. Webb-Johnson, J. Corbett, D. McManus, M. J. Krowka, T. Zeiger, U. B. Prakash, T. Staats, C. Strange, M. Baumann, M. Judson, R. Oser, N. T. Soskel, V. Smith, D. N. Killian, W. Demicco, L. Golden, R. Hitchcock, J. Moss, S. C. Chu, N. G. McElvaney, P. Barnes, K. O'Neil, D. Holden, B. M. Meth, R. W. Ashburn, J. Forrester, R. F. Sarlin, R. S. Sen, T. E. Walsh, S. Jones, J. Drake

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394 Scopus citations


Subjects ≤ 18 yr of age with serum α1-antitrypsin (α1-AT) levels ≤ 11 μM or a ZZ genotype were followed for 3.5 to 7 yr with spirometry measurements every 6 to 12 mo as part of a National Heart, Lung, and Blood Institute Registry of Patients with Severe Deficiency of Alpha-1-Antitrypsin. Among all 1,129 enrollees, 5-yr mortality was 19% (95% CI: 16 to 21%). In multivariate analyses of 1,048 subjects who had been contacted ≤ 6 mo after enrolling, age and baseline FEV1% predicted were significant predictors of mortality. Results also showed that those subjects receiving augmentation therapy. Among 927 subjects with two or more FEV1 measurements ≤ 1 yr apart, the mean FEV1 decline was 54 ml/yr, with more rapid decline in males, those aged 30 to 44 yr, current smokers, those with FEV1, 35 to 79% predicted, and those who ever had a bronchodilator response. Among all subjects, FEV1, decline was not different between augmentation-therapy groups (p=0.40). However, among subjects with a mean FEV1 35 to 49% predicted, FEV1 decline was significantly slower for subjects receiving than for those not receiving augmentation therapy (mean difference = 27 ml/yr, 95% CI: 3 to 51 ml/yr; p = 0.03). Because this was not a randomized trial, we cannot exclude the possibility that these differences may have been due to other factors for which we could not control.

Original languageEnglish (US)
Pages (from-to)49-59
Number of pages11
JournalAmerican journal of respiratory and critical care medicine
Issue number1
StatePublished - 1998

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine


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