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Pasteurized C1 inhibitor concentrate in hereditary angioedema: pharmacology, safety, efficacy and future directions

Pages 13-20 | Published online: 10 Jan 2014

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Saw Yen Ow, Eugene A. Kapp, Vesna Tomasetig, Anton Zalewski, Jason Simmonds, Con Panousis, Michael J. Wilson, Andrew D. Nash & Matthias Pelzing. (2023) HDX-MS study on garadacimab binding to activated FXII reveals potential binding interfaces through differential solvent exposure. mAbs 15:1.
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Dmitrii V. Kalinin. (2021) Factor XII(a) inhibitors: a review of the patent literature. Expert Opinion on Therapeutic Patents 31:12, pages 1155-1176.
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Iman H Nasr, Ania L Manson, Humaid A Al Wahshi & Hilary J Longhurst. (2016) Optimizing hereditary angioedema management through tailored treatment approaches. Expert Review of Clinical Immunology 12:1, pages 19-31.
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Roberto Castelli, Andrea Zanichelli & Massimo Cugno. (2013) Therapeutic options for patients with angioedema due to C1-inhibitor deficiencies: from pathophysiology to the clinic. Immunopharmacology and Immunotoxicology 35:1, pages 181-190.
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Mark M Gompels & Robert John Lock. (2011) Cinryze™ (C1-inhibitor) for the treatment of hereditary angioedema. Expert Review of Clinical Immunology 7:5, pages 569-573.
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Lilian Varga & Henriette Farkas. (2008) Treatment of type I and II hereditary angioedema with Rhucin®, a recombinant human C1 inhibitor. Expert Review of Clinical Immunology 4:6, pages 653-661.
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Articles from other publishers (27)

Andrew McKenzie, Anthony Roberts, Sourabh Malandkar, Henrike Feuersenger, Con Panousis & Dipti Pawaskar. (2021) A phase I, first‐in‐human, randomized dose‐escalation study of anti‐activated factor XII monoclonal antibody garadacimab. Clinical and Translational Science 15:3, pages 626-637.
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Konrad Bork, Emel Aygören-Pürsün, Murat Bas, Tilo Biedermann, Jens Greve, Karin Hartmann, Markus Magerl, Inmaculada Martinez-Saguer, Marcus Maurer, Hagen Ott, Lucia Schauf, Petra Staubach & Bettina Wedi. (2019) Guideline: Hereditary angioedema due to C1 inhibitor deficiency. Allergo Journal International 28:1, pages 16-29.
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Konrad Bork, Emel Aygören-Pürsün, Murat Bas, Tilo Biedermann, Jens Greve, Karin Hartmann, Markus Magerl, Inmaculada Martinez-Saguer, Marcus Maurer, Hagen Ott, Lucia Schauf, Petra Staubach & Bettina Wedi. (2019) Leitlinie: Hereditäres Angioödem durch C1-Inhibitor-Mangel. Allergo Journal 28:1, pages 31-47.
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Helen Cao, Mark Biondo, Hadi Lioe, Samantha Busfield, Veronika Rayzman, Bernhard Nieswandt, Konrad Bork, Leonard C. Harrison, Priscilla Auyeung, Henriette Farkas, Dorottya Csuka, Matthias Pelzing, Steve Dower, Michael J. Wilson, Andrew Nash, Marc W. Nolte & Con Panousis. (2018) Antibody-mediated inhibition of FXIIa blocks downstream bradykinin generation. Journal of Allergy and Clinical Immunology 142:4, pages 1355-1358.
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K. BORK, M. MAURER, M. BAS, K. HARTMANN, T. BIEDERMANN, W. KREUZ, E. AYGÖREN-PÜRSÜN, I. MARTINEZ-SAGUER, H. OTT & B. WEDI. 2015. Leitlinien Kinder- und Jugendmedizin. Leitlinien Kinder- und Jugendmedizin G3.1 G3.14 .
D. Moldovan, A. Reshef, J. Fabiani, S. Kivity, E. Toubi, M. Shlesinger, M. Triggiani, V. Montinaro, E. Cillari, G. Realdi, M. Cancian, S. Visscher, A. Zanichelli, A. Relan & M. Cicardi. (2023) Efficacy and safety of recombinant human C1‐inhibitor for the treatment of attacks of hereditary angioedema: European open‐label extension study. Clinical & Experimental Allergy 42:6, pages 929-935.
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Konrad Bork, Marcus Maurer, Murat Bas, Karin Mücke, Tilo Biedermann, Wolfhart Kreuz, Emel Aygören-Pürsün, Inmaculada Martinez-Saguer, Hagen Ott & Bettina Wedi. (2012) Hereditäres Angioödem durch C1-Inhibitor-Mangel. Allergo Journal 21:2, pages 109-120.
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Wolfhart Kreuz, Eva Rusicke, Inmaculada Martinez-Saguer, Emel Aygören-Pürsün, Christine Heller & Thomas Klingebiel. (2012) Home therapy with intravenous human C1-inhibitor in children and adolescents with hereditary angioedema. Transfusion 52:1, pages 100-107.
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Tom Bowen. (2011) Hereditary angioedema: beyond international consensus - circa December 2010 - The Canadian Society of Allergy and Clinical Immunology Dr. David McCourtie Lecture. Allergy, Asthma & Clinical Immunology 7:1.
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T. J. Craig, A. K. Bewtra, S. L. Bahna, D. Hurewitz, L. C. Schneider, R. J. Levy, J. N. Moy, J. Offenberger, K. W. Jacobson, W. H. Yang, F. Eidelman, G. Janss, F. R. Packer, M. A. Rojavin, T. Machnig, H.-O. Keinecke & R. L. Wasserman. (2011) C1 esterase inhibitor concentrate in 1085 Hereditary Angioedema attacks - final results of the I.M.P.A.C.T.2 study. Allergy 66:12, pages 1604-1611.
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Konrad Bork, Jochen Hardt, Petra Staubach-Renz & Guenther Witzke. (2011) Risk of laryngeal edema and facial swellings after tooth extraction in patients with hereditary angioedema with and without prophylaxis with C1 inhibitor concentrate: a retrospective study. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology 112:1, pages 58-64.
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Richard G. Gower, Paula J. Busse, Emel Aygören-Pürsün, Amin J. Barakat, Teresa Caballero, Mark Davis-Lorton, Henriette Farkas, David S. Hurewitz, Joshua S. Jacobs, Douglas T. Johnston, William Lumry & Marcus Maurer. (2011) Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies. World Allergy Organization Journal 4:2, pages S9-S21.
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Solange Oliveira Rodrigues ValleAlfeu Tavares FrançaRegis A. CamposAnete Sevciovic Grumach. (2011) C1 Esterase Inhibitor (Human) for the Treatment of Acute Hereditary Angioedema. Clinical medicine. Blood disorders 4, pages CMBD.S4090.
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Richard G Gower, Paula J Busse, Emel Aygören-Pürsün, Amin J Barakat, Teresa Caballero, Mark Davis-Lorton, Henriette Farkas, David S Hurewitz, Joshua S Jacobs, Douglas T Johnston, William Lumry & Marcus Maurer. (2011) Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies. World Allergy Organization Journal 4, pages S9-S21.
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Richard L. Wasserman, Robyn J. Levy, Againdra K. Bewtra, David Hurewitz, Timothy J. Craig, Peter C. Kiessling, Heinz-Otto Keinecke & Jonathan A. Bernstein. (2011) Prospective study of C1 esterase inhibitor in the treatment of successive acute abdominal and facial hereditary angioedema attacks. Annals of Allergy, Asthma & Immunology 106:1, pages 62-68.
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Hilary J Longhurst, Henriette Farkas, Timothy Craig, Emel Aygören-Pürsün, Claire Bethune, Janne Bjorkander, Konrad Bork, Laurence Bouillet, Henrik Boysen, Anette Bygum, Teresa Caballero, Marco Cicardi, John Dempster, Mark Gompels, Jimmy Gooi, Sofia Grigoriadou, Ursula Huffer, Wolfhart Kreuz, Marcel M Levi, Janet Long, Inmaculada Martinez-Saguer, Michel Raguet, Avner Reshef, Tom Bowen & Bruce Zuraw. (2010) HAE international home therapy consensus document. Allergy, Asthma & Clinical Immunology 6:1.
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Emel Aygören-Pürsün, Inmaculada Martinez-Saguer, Eva Rusicke, Thomas Klingebiel & Wolfhart Kreuz. (2010) On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience. Allergy, Asthma & Clinical Immunology 6:1.
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Timothy J. Craig, Richard L. Wasserman, Robyn J. Levy, Againdra K. Bewtra, Lynda Schneider, Flint Packer, William H. Yang, Heinz-Otto Keinecke & Peter C. Kiessling. (2010) Prospective Study of Rapid Relief Provided by C1 Esterase Inhibitor in Emergency Treatment of Acute Laryngeal Attacks in Hereditary Angioedema. Journal of Clinical Immunology 30:6, pages 823-829.
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Jonathan A. Bernstein, Bruce Ritchie, Robyn J. Levy, Richard L. Wasserman, Againdra K. Bewtra, David S. Hurewitz, Krystyna Obtulowicz, Avner Reshef, Dumitru Moldovan, Todor Shirov, Vesna Grivcheva-Panovska, Peter C. Kiessling, Fritz Schindel & Timothy J. Craig. (2010) Population pharmacokinetics of plasma-derived C1 esterase inhibitor concentrate used to treat acute hereditary angioedema attacks. Annals of Allergy, Asthma & Immunology 105:2, pages 149-154.
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Inmaculada Martinez-Saguer, Eva Rusicke, Emel Aygören-Pürsün, Christine Heller, Thomas Klingebiel & Wolfhart Kreuz. (2010) Characterization of acute hereditary angioedema attacks during pregnancy and breast-feeding and their treatment with C1 inhibitor concentrate. American Journal of Obstetrics and Gynecology 203:2, pages 131.e1-131.e7.
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Konrad Bork. (2010) Recurrent Angioedema and the Threat of Asphyxiation. Deutsches Ärzteblatt international.
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Inmaculada Martinez-Saguer, Eva Rusicke, Emel Aygören-Pürsün, Nils von Hentig, Thomas Klingebiel & Wolfhart Kreuz. (2010) Pharmacokinetic analysis of human plasma-derived pasteurized C1-inhibitor concentrate in adults and children with hereditary angioedema: a prospective study. Transfusion 50:2, pages 354-360.
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Gillian M. Keating. (2009) Human C1-Esterase Inhibitor Concentrate (Berinert®). BioDrugs 23:6, pages 399-406.
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Timothy J. Craig, Robyn J. Levy, Richard L. Wasserman, Againdra K. Bewtra, David Hurewitz, Krystyna Obtułowicz, Avner Reshef, Bruce Ritchie, Dumitru Moldovan, Todor Shirov, Vesna Grivcheva-Panovska, Peter C. Kiessling, Heinz-Otto Keinecke & Jonathan A. Bernstein. (2009) Efficacy of human C1 esterase inhibitor concentrate compared with placebo in acute hereditary angioedema attacks. Journal of Allergy and Clinical Immunology 124:4, pages 801-808.
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Wolfhart Kreuz, Inmaculada Martinez-Saguer, Emel Aygören-Pürsün, Eva Rusicke, Christine Heller & Thomas Klingebiel. (2009) C1-inhibitor concentrate for individual replacement therapy in patients with severe hereditary angioedema refractory to danazol prophylaxis. Transfusion 49:9, pages 1987-1995.
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Konrad Bork & Jochen Hardt. (2009) Hereditary Angioedema: Increased Number of Attacks after Frequent Treatments with C1 Inhibitor Concentrate. The American Journal of Medicine 122:8, pages 780-783.
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S Grigoriadou & H J Longhurst. (2009) Clinical Immunology Review Series: An approach to the patient with angio-oedema. Clinical and Experimental Immunology 155:3, pages 367-377.
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