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ORIGINAL RESEARCH

Safety and Pharmacokinetics of 120 mg/kg versus 60 mg/kg Weekly Intravenous Infusions of Alpha-1 Proteinase Inhibitor in Alpha-1 Antitrypsin Deficiency: A Multicenter, Randomized, Double-Blind, Crossover Study (SPARK)

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Pages 687-695 | Published online: 17 Jul 2013

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Read on this site (8)

Felix J F Herth, Robert A Sandhaus, Alice M Turner, Maria Sucena, Tobias Welte & Timm Greulich. (2021) Alpha 1 Antitrypsin Therapy in Patients with Alpha 1 Antitrypsin Deficiency: Perspectives from a Registry Study and Practical Considerations for Self-Administration During the COVID-19 Pandemic. International Journal of Chronic Obstructive Pulmonary Disease 16, pages 2983-2996.
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Priya S Chukowry, Ross Gareth Edgar & Alice M Turner. (2019) Alpha 1 antitrypsin deficiency: a rare multisystem disease, predominantly affecting the lung. Expert Opinion on Orphan Drugs 7:7-8, pages 315-326.
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Kenneth R Chapman, Joanna Chorostowska-Wynimko, A Rembert Koczulla, Ilaria Ferrarotti & Noel G McElvaney. (2018) Alpha 1 antitrypsin to treat lung disease in alpha 1 antitrypsin deficiency: recent developments and clinical implications. International Journal of Chronic Obstructive Pulmonary Disease 13, pages 419-432.
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Alan F Barker, Michael A Campos, Mark L Brantly, James M Stocks, Robert A Sandhaus, Douglas Lee, Kimberly Steinmann, Jiang Lin & Susan Sorrells. (2017) Bioequivalence of a Liquid Formulation of Alpha1-Proteinase Inhibitor Compared with Prolastin®-C (Lyophilized Alpha1-PI) in Alpha1-Antitrypsin Deficiency. COPD: Journal of Chronic Obstructive Pulmonary Disease 14:6, pages 590-596.
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Ross G Edgar, Mitesh Patel, Susan Bayliss, Diana Crossley, Elizabeth Sapey & Alice M Turner. (2017) Treatment of lung disease in alpha-1 antitrypsin deficiency: a systematic review. International Journal of Chronic Obstructive Pulmonary Disease 12, pages 1295-1308.
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Maria Paula Henao & Timothy J. Craig. (2016) Recent advances in understanding and treating COPD related to α1-antitrypsin deficiency. Expert Review of Respiratory Medicine 10:12, pages 1281-1294.
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Reena Berman, Di Jiang, Qun Wu & Hong Wei Chu. (2016) α1-Antitrypsin reduces rhinovirus infection in primary human airway epithelial cells exposed to cigarette smoke. International Journal of Chronic Obstructive Pulmonary Disease 11, pages 1279-1286.
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Antonio Anzueto. (2015) Alpha-1 Antitrypsin Deficiency-Associated Chronic Obstructive Pulmonary Disease: A Family Perspective. COPD: Journal of Chronic Obstructive Pulmonary Disease 12:4, pages 462-467.
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Articles from other publishers (37)

Zhaoyang Li, Mitali Gaurav & Leman Yel. (2023) Evaluation of body weight–based dosing, alternative dosing regimens, and treatment interruptions for α1-proteinase inhibitors and implications on biochemical efficacy in patients with α1-antitrypsin deficiency. Pulmonary Pharmacology & Therapeutics 83, pages 102265.
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Kuniaki Seyama, Masaru Suzuki, Sadatomo Tasaka, Toshihiro Nukiwa, Tadashi Sato, Satoshi Konno, Susan Sorrells, Junliang Chen, Maria Esperança Aragonés & Hitoshi Minamino. (2022) Long-term safety of Prolastin®-C, an alpha1-proteinase inhibitor, in Japanese patients with alpha1-antitrypsin deficiency. Respiratory Investigation 60:6, pages 831-839.
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J.-F. Mornex, M. Balduyck, M. Bouchecareilh, A. Cuvelier, R. Epaud, M. Kerjouan, O. Le Rouzic, C. Pison, L. Plantier, M.-C. Pujazon, M. Reynaud-Gaubert, A. Toutain, B. Trumbic, M.-C. Willemin, M. Zysman, O. Brun, M. Campana, F. Chabot, V. Chamouard, M. Dechomet, J. Fauve, B. Girerd, C. Gnakamene, S. Lefrançois, J.-N. Lombard, B. Maitre, C. Maynié-François, A. Moerman, A. Payancé, P. Reix, D. Revel, M.-P. Revel, M. Schuers, P. Terrioux, D. Theron, F. Willersinn, V. Cottin & H. Mal. (2022) Atteinte pulmonaire du déficit en alpha-1 antitrypsine. Recommandations pratiques pour le diagnostic et la prise en charge. Revue des Maladies Respiratoires 39:7, pages 633-656.
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Izel Koyuturk, Surbhi Kedia, Anna Robotham, Alexandra Star, Denis Brochu, Janelle Sauvageau, John Kelly, Michel Gilbert & Yves Durocher. (2022) High‐level production of wild‐type and oxidation‐resistant recombinant alpha‐1‐antitrypsin in glycoengineered CHO cells. Biotechnology and Bioengineering 119:9, pages 2331-2344.
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Lukas Wettstein, Frank Kirchhoff & Jan Münch. (2022) The Transmembrane Protease TMPRSS2 as a Therapeutic Target for COVID-19 Treatment. International Journal of Molecular Sciences 23:3, pages 1351.
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Martina Oriano, Francesco Amati, Andrea Gramegna, Anthony De Soyza, Marco Mantero, Oriol Sibila, Sanjay H. Chotirmall, Antonio Voza, Paola Marchisio, Francesco Blasi & Stefano Aliberti. (2021) Protease–Antiprotease Imbalance in Bronchiectasis. International Journal of Molecular Sciences 22:11, pages 5996.
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Lukas Wettstein, Tatjana Weil, Carina Conzelmann, Janis A. Müller, Rüdiger Groß, Maximilian Hirschenberger, Alina Seidel, Susanne Klute, Fabian Zech, Caterina Prelli Bozzo, Nico Preising, Giorgio Fois, Robin Lochbaum, Philip Maximilian Knaff, Volker Mailänder, Ludger Ständker, Dietmar Rudolf Thal, Christian Schumann, Steffen Stenger, Alexander Kleger, Günter Lochnit, Benjamin Mayer, Yasser B. Ruiz-Blanco, Markus Hoffmann, Konstantin M. J. Sparrer, Stefan Pöhlmann, Elsa Sanchez-Garcia, Frank Kirchhoff, Manfred Frick & Jan Münch. (2021) Alpha-1 antitrypsin inhibits TMPRSS2 protease activity and SARS-CoV-2 infection. Nature Communications 12:1.
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Khushboo Goel, Charlie Strange, Robert A Sandhaus, Irina Petrache & Karina A Serban. (2021) Updates in the Management of Alpha-1 Antitrypsin Deficiency Lung Disease. US Respiratory & Pulmonary Diseases 6:1, pages 26.
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Igor Barjaktarevic & Michael Campos. (2021) Management of lung disease in alpha-1 antitrypsin deficiency: what we do and what we do not know. Therapeutic Advances in Chronic Disease 12_suppl, pages 204062232110101.
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Roxana Cortes-Lopez & Igor Barjaktarevic. (2020) Alpha-1 Antitrypsin Deficiency: a Rare Disease?. Current Allergy and Asthma Reports 20:9.
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José Luis López-Campos, Laura Carrasco Hernandez & Candelaria Caballero Eraso. (2020) Implications of a Change of Paradigm in Alpha1 Antitrypsin Deficiency Augmentation Therapy: From Biochemical to Clinical Efficacy. Journal of Clinical Medicine 9:8, pages 2526.
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Benjamin M. Scott & William P. Sheffield. (2019) Engineering the serpin α 1 ‐antitrypsin: A diversity of goals and techniques . Protein Science 29:4, pages 856-871.
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Trevor Cickovski, Astrid Manuel, Kalai Mathee, Michael Campos & Giri Narasimhan. 2020. Computational Advances in Bio and Medical Sciences. Computational Advances in Bio and Medical Sciences 90 101 .
Diana Crossley, Robert Stockley & Elizabeth Sapey. (2019) Alpha-1 Antitrypsin Deficiency and Accelerated Aging: A New Model for an Old Disease?. Drugs & Aging 36:9, pages 823-840.
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Irina Petrache. (2019) Is More Better? Promising Biological Effects of Double-Dose Alpha 1-Antitrypsin Therapy. American Journal of Respiratory and Critical Care Medicine 200:3, pages 270-272.
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Michael A. Campos, Patrick Geraghty, Gregory Holt, Eliana Mendes, Paul R. Newby, Shuren Ma, Landy V. Luna-Diaz, Gerard M. Turino & Robert A. Stockley. (2019) The Biological Effects of Double-Dose Alpha-1 Antitrypsin Augmentation Therapy. A Pilot Clinical Trial. American Journal of Respiratory and Critical Care Medicine 200:3, pages 318-326.
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Kuniaki Seyama, Toshihiro Nukiwa, Tadashi Sato, Masaru Suzuki, Satoshi Konno, Kazuhisa Takahashi, Masaharu Nishimura, Kimberly Steinmann, Susan Sorrells, Junliang Chen & Ken-ichi Hayashi. (2019) Safety and pharmacokinetics of Alpha-1 MP (Prolastin®-C) in Japanese patients with alpha1-antitrypsin (AAT) deficiency. Respiratory Investigation 57:1, pages 89-96.
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Timm Greulich, Jan Chlumsky, Marion Wencker, Oliver Vit, Michael Fries, Thomas Chung, Amgad Shebl, Claus Vogelmeier, Kenneth R. Chapman & Noel G. McElvaney. (2018) Safety of biweekly α 1 -antitrypsin treatment in the RAPID programme . European Respiratory Journal 52:5, pages 1800897.
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Timothy J. Craig & Maria Paula Henao. (2018) Advances in managing COPD related to α 1 -antitrypsin deficiency: An under-recognized genetic disorder . Allergy 73:11, pages 2110-2121.
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Pius Ochieng, Sridesh Nath, Reane Macarulay, Edward Eden, Abdoulaye Dabo, Michael Campos, Xian-Cheng Jiang, Robert F. Foronjy & Patrick Geraghty. (2018) Phospholipid transfer protein and alpha-1 antitrypsin regulate Hck kinase activity during neutrophil degranulation. Scientific Reports 8:1.
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Article Editorial. (2018) Diagnosis and treatment of pulmonarydisease in α1-antitrypsin deficiency: a statement of European Respiratory Society. Russian Pulmonology 28:3, pages 273-295.
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Marc Miravitlles, Asger Dirksen, Ilaria Ferrarotti, Vladimir Koblizek, Peter Lange, Ravi Mahadeva, Noel G. McElvaney, David Parr, Eeva Piitulainen, Nicolas Roche, Jan Stolk, Gabriel Thabut, Alice Turner, Claus Vogelmeier & Robert A. Stockley. (2017) European Respiratory Society statement: diagnosis and treatment of pulmonary disease in α 1 -antitrypsin deficiency . European Respiratory Journal 50:5, pages 1700610.
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