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. Sartori; S. 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

doi:10.1164/ajrccm.158.1.9712017

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

Pulmonary and Critical Care Medicine

Research output: Contribution to journal › Article › peer-review

394 Scopus citations

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 language	English (US)
Pages (from-to)	49-59
Number of pages	11
Journal	American journal of respiratory and critical care medicine
Volume	158
Issue number	1
DOIs	https://doi.org/10.1164/ajrccm.158.1.9712017
State	Published - 1998

ASJC Scopus subject areas

Pulmonary and Respiratory Medicine
Critical Care and Intensive Care Medicine

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10.1164/ajrccm.158.1.9712017

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Vreim, C. E., Wu, M., Crystal, R. G., Buist, A. S., 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., ... 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. https://doi.org/10.1164/ajrccm.158.1.9712017

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, 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. https://doi.org/10.1164/ajrccm.158.1.9712017

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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.",

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

year = "1998",

doi = "10.1164/ajrccm.158.1.9712017",

language = "English (US)",

volume = "158",

pages = "49--59",

journal = "American journal of respiratory and critical care medicine",

issn = "1073-449X",

publisher = "American Thoracic Society",

number = "1",

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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. S.

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, 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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ER -

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

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