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Select item 70789131.

Diagnosis and treatment of lung disease associated with alpha one‐antitrypsin deficiency: A position statement from the Thoracic Society of Australia and New Zealand*

Jack Dummer, Claudia C. Dobler, Mark Holmes, Daniel Chambers, Ian A. Yang, Lianne Parkin, Sheree Smith, Peter Wark, Anouk Dev, Sandra Hodge, Eli Dabscheck, Julian Gooi, Sameh Samuel, Steven Knowles, Anne E. Holland

Respirology. 2020 Mar; 25(3): 321–335. Published online 2020 Feb 6. doi: 10.1111/resp.13774

PMCID:: PMC7078913

Abstract Article PDF–770K

Select item 57974722.

Alpha 1 antitrypsin to treat lung disease in alpha 1 antitrypsin deficiency: recent developments and clinical implications

Kenneth R Chapman, Joanna Chorostowska-Wynimko, A Rembert Koczulla, Ilaria Ferrarotti, Noel G McElvaney

Int J Chron Obstruct Pulmon Dis. 2018; 13: 419–432. Published online 2018 Jan 31. doi: 10.2147/COPD.S149429

PMCID:: PMC5797472

Abstract Article PDF–808K

Select item 55567623.

The Diagnosis and Management of Alpha-1 Antitrypsin Deficiency in the Adult

Robert A. Sandhaus, Gerard Turino, Mark L. Brantly, Michael Campos, Carroll E. Cross, Kenneth Goodman, D. Kyle Hogarth, Shandra L. Knight, James M. Stocks, James K. Stoller, Charlie Strange, Jeffrey Teckman

Chronic Obstr Pulm Dis. 2016; 3(3): 668–682. Published online 2016 Jun 6. doi: 10.15326/jcopdf.3.3.2015.0182

PMCID:: PMC5556762

Abstract Article PDF–266K

Select item 55569124.

Serum Proteins Associated with Emphysema Progression in Severe Alpha-1 Antitrypsin Deficiency

Tatsiana Beiko, Michael G. Janech, Alexander V. Alekseyenko, Carl Atkinson, Harvey O. Coxson, Jeremy L. Barth, Sarah E. Stephenson, Carole L. Wilson, Lynn M. Schnapp, Alan Barker, Mark Brantly, Robert A. Sandhaus, Edwin K. Silverman, James K. Stoller, Bruce Trapnell, Charlie Strange, for QUANTUM-1 Investigators

Chronic Obstr Pulm Dis. 2017; 4(3): 204–216. Published online 2017 Jul 15. doi: 10.15326/jcopdf.4.3.2016.0180

PMCID:: PMC5556912

Abstract Article PDF–4.1M

Select item 55569685.

Characteristics of Chronic Obstructive Pulmonary Disease (COPD) Patients Reporting Alpha-1 Antitrypsin Deficiency in the WebMD Lung Health Check Database

Tyler McGrady, David M. Mannino, Elisha Malanga, Byron M. Thomashow, John Walsh, Robert A. Sandhaus, James K. Stoller

Chronic Obstr Pulm Dis. 2015; 2(2): 141–151. Published online 2015 Apr 15. doi: 10.15326/jcopdf.2.2.2014.0160

PMCID:: PMC5556968

Abstract Article PDF–6.0M

Select item 55569706.

Clinical Trial Design for Alpha-1 Antitrypsin Deficiency: A Model for Rare Diseases

Adam Wanner, Stephen C. Groft, J. Russell Teagarden, Jeffrey Krischer, Barry R. Davis, Christopher S. Coffey, David H. Hickam, Jeffrey Teckman, David R. Nelson, Michael L. McCaleb, Rohit Loomba, Charlie Strange, Robert A. Sandhaus, Mark Brantly, Jonathan M. Edelman, Albert Farrugia

Chronic Obstr Pulm Dis. 2015; 2(2): 177–190. Published online 2015 Apr 28. doi: 10.15326/jcopdf.2.2.2015.0132

PMCID:: PMC5556970

Abstract Article PDF–2.1M

Select item 55602487.

The Effect of Alpha-1 Proteinase Inhibitor on Biomarkers of Elastin Degradation in Alpha-1 Antitrypsin Deficiency: An Analysis of the RAPID/RAPID Extension Trials

Shuren Ma, Yong Y. Lin, Jerome O. Cantor, Kenneth R. Chapman, Robert A. Sandhaus, Michael Fries, Jonathan M. Edelman, Gerard McElvaney, Gerard M. Turino

Chronic Obstr Pulm Dis. 2017; 4(1): 34–44. Published online 2016 Nov 18. doi: 10.15326/jcopdf.4.1.2016.0156

PMCID:: PMC5560248

Abstract Article PDF–2.3M

Select item 55602508.

Benefits Among Patients with Alpha-1 Antitrypsin Deficiency Enrolled in a Disease Management and Prevention Program

Jordan T. Perkins, Radmila Choate, David M. Mannino, Stephen R. Browning, Robert A. Sandhaus

Chronic Obstr Pulm Dis. 2017; 4(1): 56–64. Published online 2016 Dec 24. doi: 10.15326/jcopdf.4.1.2016.0161

PMCID:: PMC5560250

Abstract Article PDF–6.1M

Select item 56029619.

Cardiovascular risk in patients with alpha-1-antitrypsin deficiency

Sebastian Fähndrich, Frank Biertz, Annika Karch, Björn Kleibrink, Armin Koch, Helmut Teschler, Tobias Welte, Hans-Ulrich Kauczor, Sabina Janciauskiene, Rudolf A. Jörres, Timm Greulich, Claus F. Vogelmeier, Robert Bals, on behalf of the COSYCONET investigators

Respir Res. 2017; 18: 171. Published online 2017 Sep 15. doi: 10.1186/s12931-017-0655-1

PMCID:: PMC5602961

Abstract Article PDF–921K

Select item 563783210.

Should Serum Protein Electrophoresis Be a Surrogate for Liver Biopsy in Some Cases of Alpha₁ Antitrypsin Deficiency?

Newton Key Hokama, Marcelo Padovani de Toledo Moraes, Paula de Oliveira Montandon Hokama, Fernando Gomes Romeiro

Case Reports Hepatol. 2017; 2017: 2705131. Published online 2017 Sep 28. doi: 10.1155/2017/2705131

PMCID:: PMC5637832

Abstract Article PDF–1.4M

Select item 565131311.

Quantitative disease progression model of α‐1 proteinase inhibitor therapy on computed tomography lung density in patients with α‐1 antitrypsin deficiency

Michael A. Tortorici, James A. Rogers, Oliver Vit, Martin Bexon, Robert A. Sandhaus, Jonathan Burdon, Joanna Chorostowska‐Wynimko, Philip Thompson, James Stocks, Noel G. McElvaney, Kenneth R. Chapman, Jonathan M. Edelman

Br J Clin Pharmacol. 2017 Nov; 83(11): 2386–2397. Published online 2017 Aug 11. doi: 10.1111/bcp.13358

PMCID:: PMC5651313

Abstract Article PDF–970K

Select item 568378312.

Lung transplantation and survival outcomes in patients with oxygen-dependent COPD with regard to their alpha-1 antitrypsin deficiency status

Magnus Ekström, Hanan Tanash

Int J Chron Obstruct Pulmon Dis. 2017; 12: 3281–3287. Published online 2017 Nov 7. doi: 10.2147/COPD.S148509

PMCID:: PMC5683783

Abstract Article PDF–397K

Select item 571030713.

Diagnosing alpha-1 antitrypsin deficiency: the first step in precision medicine

Craig P. Hersh

Version 1. F1000Res. 2017; 6: 2049. Published online 2017 Nov 27. doi: 10.12688/f1000research.12399.1

PMCID:: PMC5710307

Abstract Article PDF–1.2M

Select item 572756814.

Successful Outcome and Biliary Drainage in an Infant with Concurrent Alpha-1-Antitrypsin Deficiency and Biliary Atresia

Andrew W. Wang, Kimberly Newton, Karen Kling

Case Rep Surg. 2017; 2017: 9348461. Published online 2017 Nov 29. doi: 10.1155/2017/9348461

PMCID:: PMC5727568

Abstract Article PDF–1.8M

Select item 574526215.

Survival after Lung Transplantation in Recipients with Alpha-1-Antitrypsin Deficiency Compared to Other Forms of Chronic Obstructive Pulmonary Disease: A National Cohort Study

Brian C Gulack, Michael S Mulvihill, Asvin M Ganapathi, Paul J Speicher, Godefroy Chery, Laurie D Snyder, R Duane Davis, Matthew G Hartwig

Transpl Int. Author manuscript; available in PMC 2019 Jan 1.

Published in final edited form as: Transpl Int. 2018 Jan; 31(1): 45–55. Published online 2017 Sep 12. doi: 10.1111/tri.13038

PMCID:: PMC5745262

Abstract Article PDF–404K

Select item 578054316.

Liver Disease in Alpha-1 Antitrypsin Deficiency: Current Approaches and Future Directions

Ellen L. Mitchell, Zahida Khan

Curr Pathobiol Rep. 2017; 5(3): 243–252. Published online 2017 Jul 10. doi: 10.1007/s40139-017-0147-5

Correction in:: Curr Pathobiol Rep. 2018; 6(1): 97.

PMCID:: PMC5780543

Abstract Article PDF–914K

Select item 552911117.

Clinical utility of alpha-1 proteinase inhibitor in the management of adult patients with severe alpha-1 antitrypsin deficiency: a review of the current literature

David G Parr, Beatriz Lara

Drug Des Devel Ther. 2017; 11: 2149–2162. Published online 2017 Jul 14. doi: 10.2147/DDDT.S105207

PMCID:: PMC5529111

Abstract Article PDF–621K

Select item 587063718.

Long-term evolution of lung function in individuals with alpha-1 antitrypsin deficiency from the Spanish registry (REDAAT)

Cristina Esquinas, Sonia Serreri, Miriam Barrecheguren, Esther Rodriguez, Alexa Nuñez, Francisco Casas-Maldonado, Ignacio Blanco, Pietro Pirina, Beatriz Lara, Marc Miravitlles

Int J Chron Obstruct Pulmon Dis. 2018; 13: 1001–1007. Published online 2018 Mar 23. doi: 10.2147/COPD.S155226