Am. J. Respir. Crit. Care Med.,
Volume 158, Number 1, July 1998, 49-59
 1-Antitrypsin
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18 yr of age with serum 
1-antitrypsin (
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  |
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  M. Griese and H. Bruggen Intravenous {alpha}1-antitrypsin in a child with deficiency and severe lung disease Eur. Respir. J., July 1, 2009; 34(1): 278 - 279. [Full Text] [PDF]
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 E. K. Silverman and R. A. Sandhaus Alpha1-Antitrypsin Deficiency N. Engl. J. Med., June 25, 2009; 360(26): 2749 - 2757. [Full Text] [PDF]
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J. B. Soriano and M. Miravitlles Your racing horses will help you to quit: a lesson for COPD and {alpha}1-antitrypsin deficiency research Eur. Respir. J., June 1, 2009; 33(6): 1244 - 1246. [Full Text] [PDF]
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P. A. Dawkins, C. L. Dawkins, A. M. Wood, P. G. Nightingale, J. A. Stockley, and R. A. Stockley Rate of progression of lung function impairment in {alpha}1-antitrypsin deficiency Eur. Respir. J., June 1, 2009; 33(6): 1338 - 1344. [Abstract] [Full Text] [PDF]
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A. Dirksen, E. Piitulainen, D. G. Parr, C. Deng, M. Wencker, S. B. Shaker, and R. A. Stockley Exploring the role of CT densitometry: a randomised study of augmentation therapy in {alpha}1-antitrypsin deficiency Eur. Respir. J., June 1, 2009; 33(6): 1345 - 1353. [Abstract] [Full Text] [PDF]
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 J. K. Stoller and L. S. Aboussouan Myths and Misconceptions About {alpha}1-Antitrypsin Deficiency Arch Intern Med, March 23, 2009; 169(6): 546 - 550. [Full Text] [PDF]
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 M. A. Campos, S. Alazemi, G. Zhang, M. Salathe, A. Wanner, R. A. Sandhaus, and H. Baier Clinical Characteristics of Subjects With Symptoms of {alpha}1-Antitrypsin Deficiency Older Than 60 Years Chest, March 1, 2009; 135(3): 600 - 608. [Abstract] [Full Text] [PDF]
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 M. L. Snyder and C. R. Fantz Cirrhosis Originally Diagnosed as Nonalcoholic Steatohepatitis Clin. Chem., August 1, 2008; 54(8): 1395 - 1398. [Full Text] [PDF]
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 C. P. Hersh, D. L. DeMeo, and E. K. Silverman National Emphysema Treatment Trial State of the Art: Genetics of Emphysema Proceedings of the ATS, May 1, 2008; 5(4): 486 - 493. [Abstract] [Full Text] [PDF]
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 D. L DeMeo, R. A Sandhaus, A. F Barker, M. L Brantly, E. Eden, N G. McElvaney, S. Rennard, E. Burchard, J. M Stocks, J. K Stoller, et al. Determinants of airflow obstruction in severe alpha-1-antitrypsin deficiency Thorax, September 1, 2007; 62(9): 806 - 813. [Abstract] [Full Text] [PDF]
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 J. K. Stoller, T. R. Gildea, A. L. Ries, Y. M. Meli, M. T. Karafa, and National Emphysema Treatment Trial Research Group Lung Volume Reduction Surgery in Patients With Emphysema and {alpha}-1 Antitrypsin Deficiency Ann. Thorac. Surg., January 1, 2007; 83(1): 241 - 251. [Abstract] [Full Text] [PDF]
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J. K. Stoller, J. Tomashefski Jr, R. G. Crystal, A. Arroliga, C. Strange, D. N. Killian, M. D. Schluchter, H. P. Wiedemann, and for the α1-Antitrypsin Deficiency Registry S Mortality in Individuals With Severe Deficiency of α1-Antitrypsin: Findings From the National Heart, Lung, and Blood Institute Registry Chest, April 1, 2005; 127(4): 1196 - 1204. [Abstract] [Full Text] [PDF]
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M Needham and R A Stockley {alpha}1-Antitrypsin deficiency * 3: Clinical manifestations and natural history Thorax, May 1, 2004; 59(5): 441 - 445. [Abstract] [Full Text] [PDF]
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L. J. Dowson, P. J. Guest, and R. A. Stockley The Relationship of Chronic Sputum Expectoration to Physiologic, Radiologic, and Health Status Characteristics in {alpha}1-Antitrypsin Deficiency (PiZ) Chest, October 1, 2002; 122(4): 1247 - 1255. [Abstract] [Full Text] [PDF]
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I S Woolhouse, D L Bayley, and R A Stockley Sputum chemotactic activity in chronic obstructive pulmonary disease: effect of {alpha}1-antitrypsin deficiency and the role of leukotriene B4 and interleukin 8 Thorax, August 1, 2002; 57(8): 709 - 714. [Abstract] [Full Text] [PDF]
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J. K. Stoller, F. Rouhani, M. Brantly, S. Shahin, R. A. Dweik, J. M. Stocks, J. Clausen, E. Campbell, and F. Norton Biochemical Efficacy and Safety of a New Pooled Human Plasma {alpha}1-Antitrypsin, Respitin* Chest, July 1, 2002; 122(1): 66 - 74. [Abstract] [Full Text] [PDF]
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R. W. Carrell and D. A. Lomas Alpha1-Antitrypsin Deficiency -- A Model for Conformational Diseases N. Engl. J. Med., January 3, 2002; 346(1): 45 - 53. [Full Text] [PDF]
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M. Wencker, B. Fuhrmann, N. Banik, and N. Konietzko Longitudinal Follow-up of Patients With {{alpha}}1-Protease Inhibitor Deficiency Before and During Therapy With IV {{alpha}}1-Protease Inhibitor Chest, March 1, 2001; 119(3): 737 - 744. [Abstract] [Full Text] [PDF]
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J. K. Stoller, M. Brantly, L. E. Fleming, J. A. Bean, and J. Walsh Formation and Current Results of a Patient-Organized Registry for {alpha}1-Antitrypsin Deficiency Chest, September 1, 2000; 118(3): 843 - 848. [Abstract] [Full Text] [PDF]
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J. S. Wilson and J. R. Galvin Normal Diffusing Capacity in Patients With PiZ {alpha}1-Antitrypsin Deficiency, Severe Airflow Obstruction, and Significant Radiographic Emphysema Chest, September 1, 2000; 118(3): 867 - 871. [Abstract] [Full Text] [PDF]
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R A Stockley Alpha-1-antitrypsin deficiency: what next? Thorax, July 1, 2000; 55(7): 614 - 618. [Full Text]
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S. A. Alkins and P. O’Malley Should Health-Care Systems Pay for Replacement Therapy in Patients With {alpha}1-Antitrypsin Deficiency?: A Critical Review and Cost-Effectiveness Analysis Chest, March 1, 2000; 117(3): 875 - 880. [Abstract] [Full Text] [PDF]
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 M. D. SCHLUCHTER, J. K. STOLLER, A. F. BARKER, A. SONIA BUIST, R. G. CRYSTAL, J. F. DONOHUE, R. J. FALLAT, G. M. TURINO, C. E. VREIM, and M. C. WU Feasibility of a Clinical Trial of Augmentation Therapy for alpha 1-Antitrypsin Deficiency Am. J. Respir. Crit. Care Med., March 1, 2000; 161(3): 796 - 801. [Abstract] [Full Text] [PDF]
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 R. Dhami, B. Gilks, C. Xie, K. Zay, J. L. Wright, and A. Churg Acute Cigarette Smoke-Induced Connective Tissue Breakdown Is Mediated by Neutrophils and Prevented by alpha 1-Antitrypsin Am. J. Respir. Cell Mol. Biol., February 1, 2000; 22(2): 244 - 252. [Abstract] [Full Text]
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 M J KROWKA Hepatopulmonary syndromes Gut, January 1, 2000; 46(1): 1 - 4. [Full Text] [PDF]
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A. DIRKSEN, J. H. DIJKMAN, F. MADSEN, B. STOEL, D. C. S. HUTCHISON, C. S. ULRIK, L. T. SKOVGAARD, A. KOK-JENSEN, A. RUDOLPHUS, N. SEERSHOLM, et al. A Randomized Clinical Trial of alpha 1-Antitrypsin Augmentation Therapy Am. J. Respir. Crit. Care Med., November 1, 1999; 160(5): 1468 - 1472. [Abstract] [Full Text]
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 RATIONALE AND DESIGN OF THE NATIONAL EMPHYSEMA TREATMENT TRIAL (NETT): A PROSPECTIVE RANDOMIZED TRIAL OF LUNG VOLUME REDUCTION SURGERY J. Thorac. Cardiovasc. Surg., September 1, 1999; 118(3): 518 - 528. [Full Text] [PDF]
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S. R. JOHNSON and A. E. TATTERSFIELD Decline in Lung Function in Lymphangioleiomyomatosis . Relation to Menopause and Progesterone Treatment Am. J. Respir. Crit. Care Med., August 1, 1999; 160(2): 628 - 633. [Abstract] [Full Text]
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J K STOLLER Augmentation therapy for severe alpha 1-antitrypsin deficiency: is the jury still out on a trial? Thorax, December 1, 1998; 53(12): 1007 - 1009. [Full Text]
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