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

C. E. Vreim, M. Wu, R. G. Crystal, A (Sonia) 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 J.F., 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

Research output: Contribution to journalArticle

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Abstract

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
Volume158
Issue number1
StatePublished - 1998
Externally publishedYes

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alpha 1-Antitrypsin Deficiency
National Heart, Lung, and Blood Institute (U.S.)
Survival
Mortality
Bronchodilator Agents
Spirometry
Group Psychotherapy
Registries
Multivariate Analysis
Genotype
Therapeutics
Serum
Autosomal Recessive alpha-1-Antitrypsin Deficiency

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Vreim, C. E., Wu, M., Crystal, R. G., Buist, A. S., Burrows, B., Cohen, A. B., ... Drake, J. (1998). Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin. American Journal of Respiratory and Critical Care Medicine, 158(1), 49-59.

Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin. / Vreim, C. E.; Wu, M.; Crystal, R. G.; Buist, A (Sonia); Burrows, B.; Cohen, A. B.; Fallat, R. J.; Gadek, J. E.; Rousell, R. H.; Schwartz, R. S.; Turino, G. M.; Schluchter, M. D.; Stoller, J. K.; Wiedemann, H. P.; Williams, G. W.; Barrett, D. M.; Beck, G. J.; Midcalf, V.; Moore, B.; Sartori, P.; Sherer, S. G.; Zhang, R.; Petty, T. L.; Tomashefski J.F., Jr; Brantly, M. L.; Hildesheim, J.; Rundquist, B.; Sandhaus, R. A.; Bell, C. W.; Berend, J.; Begle, B.; Erb, D.; Seidman, J.; Sherman, S.; Cameron, B.; Weinberger, S.; Rosenberg, M.; Johnston, R.; Rosenberg, M.; Arroliga, A. C.; Meeker, D. P.; Mehta, A.; Laskowski, D.; Ryan, J.; Loftin, J. P.; Johnson, K.; Kotch, A.; Clark, T.; Epstein, P. E.; Del Buono, P.; Sandblom, R. E.; Hert, R. C.; DeMaine, J. B.; Colar, L.; Eichenhorn, M. S.; McMahan, J. P.; Breite, W. M.; Webb-Johnson, D.; Corbett, J.; McManus, D.; Krowka, M. J.; Zeiger, T.; Prakash, U. B.; Staats, T.; Strange, C.; Baumann, M.; Judson, M.; Oser, R.; Soskel, N. T.; Smith, V.; Killian, D. N.; Demicco, W.; Golden, L.; Hitchcock, R.; Moss, J.; Chu, S. C.; McElvaney, N. G.; Barnes, P.; O'Neil, K.; Holden, D.; Meth, B. M.; Ashburn, R. W.; Forrester, J.; Sarlin, R. F.; Sen, R. S.; Walsh, T. E.; Jones, S.; Drake, J.

In: American Journal of Respiratory and Critical Care Medicine, Vol. 158, No. 1, 1998, p. 49-59.

Research output: Contribution to journalArticle

Vreim, CE, Wu, M, Crystal, RG, Buist, AS, Burrows, B, Cohen, AB, Fallat, RJ, Gadek, JE, Rousell, RH, Schwartz, RS, Turino, GM, Schluchter, MD, Stoller, JK, Wiedemann, HP, Williams, GW, Barrett, DM, Beck, GJ, Midcalf, V, Moore, B, Sartori, P, Sherer, SG, Zhang, R, Petty, TL, Tomashefski J.F., J, Brantly, ML, Hildesheim, J, Rundquist, B, Sandhaus, RA, Bell, CW, Berend, J, Begle, B, Erb, D, Seidman, J, Sherman, S, Cameron, B, Weinberger, S, Rosenberg, M, Johnston, R, Rosenberg, M, Arroliga, AC, Meeker, DP, Mehta, A, Laskowski, D, Ryan, J, Loftin, JP, Johnson, K, Kotch, A, Clark, T, Epstein, PE, Del Buono, P, Sandblom, RE, Hert, RC, DeMaine, JB, Colar, L, Eichenhorn, MS, McMahan, JP, Breite, WM, Webb-Johnson, D, Corbett, J, McManus, D, Krowka, MJ, Zeiger, T, Prakash, UB, Staats, T, Strange, C, Baumann, M, Judson, M, Oser, R, Soskel, NT, Smith, V, Killian, DN, Demicco, W, Golden, L, Hitchcock, R, Moss, J, Chu, SC, McElvaney, NG, Barnes, P, O'Neil, K, Holden, D, Meth, BM, Ashburn, RW, Forrester, J, Sarlin, RF, Sen, RS, Walsh, TE, Jones, S & Drake, J 1998, 'Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin', American Journal of Respiratory and Critical Care Medicine, vol. 158, no. 1, pp. 49-59.
Vreim, C. E. ; Wu, M. ; Crystal, R. G. ; Buist, A (Sonia) ; Burrows, B. ; Cohen, A. B. ; Fallat, R. J. ; Gadek, J. E. ; Rousell, R. H. ; Schwartz, R. S. ; Turino, G. M. ; Schluchter, M. D. ; Stoller, J. K. ; Wiedemann, H. P. ; Williams, G. W. ; Barrett, D. M. ; Beck, G. J. ; Midcalf, V. ; Moore, B. ; Sartori, P. ; Sherer, S. G. ; Zhang, R. ; Petty, T. L. ; Tomashefski J.F., Jr ; Brantly, M. L. ; Hildesheim, J. ; Rundquist, B. ; Sandhaus, R. A. ; Bell, C. W. ; Berend, J. ; Begle, B. ; Erb, D. ; Seidman, J. ; Sherman, S. ; Cameron, B. ; Weinberger, S. ; Rosenberg, M. ; Johnston, R. ; Rosenberg, M. ; Arroliga, A. C. ; Meeker, D. P. ; Mehta, A. ; Laskowski, D. ; Ryan, J. ; Loftin, J. P. ; Johnson, K. ; Kotch, A. ; Clark, T. ; Epstein, P. E. ; Del Buono, P. ; Sandblom, R. E. ; Hert, R. C. ; DeMaine, J. B. ; Colar, L. ; Eichenhorn, M. S. ; McMahan, J. P. ; Breite, W. M. ; Webb-Johnson, D. ; Corbett, J. ; McManus, D. ; Krowka, M. J. ; Zeiger, T. ; Prakash, U. B. ; Staats, T. ; Strange, C. ; Baumann, M. ; Judson, M. ; Oser, R. ; Soskel, N. T. ; Smith, V. ; Killian, D. N. ; Demicco, W. ; Golden, L. ; Hitchcock, R. ; Moss, J. ; Chu, S. C. ; McElvaney, N. G. ; Barnes, P. ; O'Neil, K. ; Holden, D. ; Meth, B. M. ; Ashburn, R. W. ; Forrester, J. ; Sarlin, R. F. ; Sen, R. S. ; Walsh, T. E. ; Jones, S. ; Drake, J. / Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin. In: American Journal of Respiratory and Critical Care Medicine. 1998 ; Vol. 158, No. 1. pp. 49-59.
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title = "Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin",
abstract = "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.",
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TY - JOUR

T1 - Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin

AU - Vreim, C. E.

AU - Wu, M.

AU - Crystal, R. G.

AU - Buist, A (Sonia)

AU - Burrows, B.

AU - Cohen, A. B.

AU - Fallat, R. J.

AU - Gadek, J. E.

AU - Rousell, R. H.

AU - Schwartz, R. S.

AU - Turino, G. M.

AU - Schluchter, M. D.

AU - Stoller, J. K.

AU - Wiedemann, H. P.

AU - Williams, G. W.

AU - Barrett, D. M.

AU - Beck, G. J.

AU - Midcalf, V.

AU - Moore, B.

AU - Sartori, P.

AU - Sherer, S. G.

AU - Zhang, R.

AU - Petty, T. L.

AU - Tomashefski J.F., Jr

AU - Brantly, M. L.

AU - Hildesheim, J.

AU - Rundquist, B.

AU - Sandhaus, R. A.

AU - Bell, C. W.

AU - Berend, J.

AU - Begle, B.

AU - Erb, D.

AU - Seidman, J.

AU - Sherman, S.

AU - Cameron, B.

AU - Weinberger, S.

AU - Rosenberg, M.

AU - Johnston, R.

AU - Rosenberg, M.

AU - Arroliga, A. C.

AU - Meeker, D. P.

AU - Mehta, A.

AU - Laskowski, D.

AU - Ryan, J.

AU - Loftin, J. P.

AU - Johnson, K.

AU - Kotch, A.

AU - Clark, T.

AU - Epstein, P. E.

AU - Del Buono, P.

AU - Sandblom, R. E.

AU - Hert, R. C.

AU - DeMaine, J. B.

AU - Colar, L.

AU - Eichenhorn, M. S.

AU - McMahan, J. P.

AU - Breite, W. M.

AU - Webb-Johnson, D.

AU - Corbett, J.

AU - McManus, D.

AU - Krowka, M. J.

AU - Zeiger, T.

AU - Prakash, U. B.

AU - Staats, T.

AU - Strange, C.

AU - Baumann, M.

AU - Judson, M.

AU - Oser, R.

AU - Soskel, N. T.

AU - Smith, V.

AU - Killian, D. N.

AU - Demicco, W.

AU - Golden, L.

AU - Hitchcock, R.

AU - Moss, J.

AU - Chu, S. C.

AU - McElvaney, N. G.

AU - Barnes, P.

AU - O'Neil, K.

AU - Holden, D.

AU - Meth, B. M.

AU - Ashburn, R. W.

AU - Forrester, J.

AU - Sarlin, R. F.

AU - Sen, R. S.

AU - Walsh, T. E.

AU - Jones, S.

AU - Drake, J.

PY - 1998

Y1 - 1998

N2 - 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.

AB - 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.

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