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The Pathophysiology of Hereditary Angioedema [PDF]
World Allergy Organization Journal, 2010 Hereditary angioedema (HAE) causes recurrent episodes of angioedema that may be very severe and are frequently associated with significant morbidity and even mortality. Understanding the pathophysiology of this disease is crucial for proper diagnosis and management of these patients.openaire +5 more sourcesIcatibant, a new bradykinin-receptor antagonist, in hereditary angioedema [PDF]
, 2010 BACKGROUND: Hereditary angioedema is characterized by recurrent attacks of angioedema of the skin, larynx, and gastrointestinal tract. Bradykinin is the key mediator of symptoms. Icatibant is a selective bradykinin B2 receptor antagonist.Wing-Tze Fan, W. Yang, Shmuel Kivity, Lumry, William, Jens Greve, Feighery, C., Zimmermann, Jens, Avner Reshef, B. Ritchie, Grabbe, Jürgen, Toubi, E., Zanichelli, A., I. Kravec, Floccard, B., F. Arcoleo, Kaatz, Martin, C. Pichler, Hoffmann, Thomas K., Riedl, M., Obtulowicz, K., Robyn J. Levy, William Yang, Jonathan A. Bernstein, Cillari, Enrico, A. Banerji, T. K. Hoffmann, David Resnick, Werner, Sonja,, Sitkauskiene, Brigita, Werner, Sonja, Bernstein, J. A., Yang, William, Schmid-Grendelmeier, Peter, Marco Cicardi, Murat Bas, Jacques Hébert, Bork, Konrad, Bas, Murat, Resnick, D., Martin Kaatz, Levy, Robyn J, Marc Riedl, Kravec, I., B. Rosenkranz, Smith, W. B., Zanichelli, Andrea, Dong, L., Fernández Romero, Diego S, Henriette Farkas, Hoffmann, T. K., Bernstein, Jonathan A, Floccard, Bernard, Malbran, A., K. Rosen, Aberer, W., Francesco Arcoleo, A. Zanichelli, Kimberly Rosen, C. H. Katelaris, Yang, W., Bernd Rosenkranz, A. Malbrán, Katelaris, Constance H, Banerji, Aleena, Langton, D., Henning Bier, W. B. Smith, Bork, K., Kreuz, Wolfhart, Wombolt, Duane, Gleich, G., Fan, W. -T., P. Keith, J. Grabbe, Ritchie, B., Hans Merk, Knolle, Jochen, Suresh Anné, F. Bracho, Langton, David, Arcoleo, F., K. W. Jacobson, Bas, M., Levy, Robyn J., D. S. Fernández Romero, William Lumry, Aberer, Werner, Smith, W., Aleena Banerji, D. Hurewitz, E. Cillari, Bernard Floccard, Martin, Ludovic, Bruce Ritchie, J. A. Bernstein, D. Resnick, Constance H. Katelaris, Kaatz, M., William B. Smith, Duane Wombolt, Cicardi, M., Lumry, W, Reshef, A., Rosenkranz, B, B. Floccard, Ritchie, Bruce, Bouillet, L., E. Toubi, Gerald Gleich, Jochen Knolle, Enrico Cillari, Kivity, S., Merk, H., Conleth Feighery, Hurewitz, D., D. Langton, Keith, Paul, Hoffmann, Thomas K, Luger, Thomas, Katelaris, C. H., Sitkauskiene, B., Andrea Zanichelli, Kirkpatrick, Charles H., Björkander, Janne, Jacobson, Kraig W, Irina Kravec, et al, S. Werner, Anné, Suresh, C. Feighery, Malbrán, Alejandro, Anne, S., J. Hébert, Pichler, C., Christiane Pichler, Jens Zimmermann, Bork, K, M. Kaatz, J. Zimmermann, Greve, J., Kreuz, W., Wombolt, D., Bull, Christian, Martin, L., S. Kivity, D. Wombolt, David Hurewitz, L. Dong, David Langton, Pichler, Christiane, Kirkpatrick, C. H., Marcus Maurer, Zimmermann, J., M. Bas, Bracho, Francisco, Hébert, Jacques, J. Björkander, Cicardi, M, Brigita Sitkauskiene, Kraig W. Jacobson, Hurewitz, David, Laurence Bouillet, Katelaris, Constance H., Maurer, Marcus, Riedl, M, Lund University., Greve, Jens, Rosenkranz, Bernd, Krystyna Obtulowicz, L. Bouillet, C. Bull, Peter Schmid-Grendelmeier, Diego S. Fernández Romero, Banerji, A, Banerji, A., Lumry, W., Thomas K. Hoffmann, Sonja Werner, Hebert, J., S. Anné, Kravec, Irina, Rosen, K., Dong, Liying, M. Cicardi, Arcoleo, Francesco, W. Lumry, H. Bier, Jacobson, K. W., Rosen, Kimberly, Cillari, E., Jürgen Grabbe, Smith, William B, Bouillet, Laurence, H. Farkas, R. J. Levy, Obtulowicz, Krystyna, Fan, Wing-Tze, Resnick, David, Levy, R. J., G. Gleich, T. Luger, Bier, Henning, Bjorkander, J., Farkas, H., Feighery, Conleth, Kirkpatrick, Charles H, P. Schmid Grendelmeier, L. Martin, Bracho, F, Maurer, M., W. Aberer, Farkas, Henriette, Bier, H., Ludovic Martin, Toubi, Elias, Keith, P., A. Reshef, Janne Björkander, Gleich, Gerald, Knolle, J., B. Sitkauskiene, Konrad Bork, Bernstein, Jonathan A., Malbrán, A, Riedl, Marc, Kivity, Shmuel, Smith, William B., J. Greve, Cicardi, Marco, Christian Bull, Rosenkranz, B., Luger, T., Elias Toubi, H. Merk, Merk, Hans, Liying Dong, Werner Aberer, J. Knolle, W. T. Fan, Reshef, Avner, Bracho, F., Francisco Bracho, K. Obtulowicz, C. H. Kirkpatrick, Fernandez Romero, D. S., Thomas Luger, Charles H. Kirkpatrick, Bull, C., Grabbe, J., W. Kreuz, Jacobson, Kraig W., Alejandro Malbrán, Schmid-Grendelmeier, P., K. Bork, M. Maurer, Paul Keith, Fernández Romero, Diego S., M. Riedl, Wolfhart Kreuz +258 morecore +1 more sourceSwitching Long‐Term Prophylaxis to Donidalorsen for Hereditary Angioedema: 1‐Year OASISplus Results
Allergy, EarlyView.This study evaluated the long‐term safety and efficacy of donidalorsen in patients who switched from a long‐term prophylactic treatment (LTP) to donidalorsen with 1‐year outcomes. Patients who switched from LTP to donidalorsen experienced a 67.6% reduction in HAE attack rates over 52 weeks.Marc A. Riedl, Jonathan A. Bernstein, Joshua S. Jacobs, Timothy Craig, William R. Lumry, H. James Wedner, Aleena Banerji, Selina Gierer, Andrew Smith, Michael E. Manning, Francesca Perego, Laura Bordone, Sabrina Treadwell, Tao Lin, Kenneth B. Newman, Aaron Yarlas, Danny M. Cohn +16 morewiley +1 more sourceModeling Hereditary Angioedema With Personalized EPSC‐Derived Hepatocytes: A CRISPR‐Validated Platform for Mutation‐Specific Mechanisms and Therapeutic Innovation
Allergy, EarlyView.Patient‐derived expanded potential stem cell (EPSC) hepatocytes reveal that pathogenic SERPING1 variants cause distinct cellular defects in hereditary angioedema. While most mutations reduce SERPING1 transcription and C1‐INH secretion, a large deletion induces intracellular C1‐INH retention.Xueyan Liu, Yuxin Wang, Jane C. Y. Wong, Xiang Zhang, Cuixi Wu, Pengyu Chen, Yunzhi Yang, Pentao Liu, Chak Sing Lau, Matthew Cook, Philip Hei Li +10 morewiley +1 more sourceMaternal Prebiotic Supplementation Modifies Associations Between Intrapartum Antibiotics and Infant Allergy
Allergy, EarlyView.This study examined whether antibiotic use altered the risk of infant allergic disease within a maternal prebiotics supplementation trial. Intrapartum antibiotics were maternal antibiotics administered during childbirth, from the onset of labour or induction until 1‐h before delivery.Summer V. M. Walker, Thomas R. Sullivan, Rachelle A. Pretorius, Nina D'Vaz, Johnny Lo, Johan Garssen, Jeffrey A. Keelan, Desiree Silva, Susan L. Prescott, Claus T. Christophersen, Debra J. Palmer +10 morewiley +1 more sourceHEREDITARY ANGIOEDEMA: AN UPDATE. [PDF]
, 2015 Objective: Hereditary angioedema is a genetic disease with autosomal dominance inheritance characterized by quantitative and qualitative deficiency of C1 inhibitor, resulting in edema of multiples organs.Thomaz, José Eduardo, Chong Neto, Herberto José, Machado, Ana Flávia, Riedi, Carlos Antônio, Rosário, Nelson Augusto +4 morecore +2 more sourcesBasophils in Immunity: Activation Pathways, Roles in Allergy and AllergoOncology and Application of the Basophil Activation Test
Allergy, EarlyView.ABSTRACT
Basophils, the least abundant leukocytes, are increasingly recognised as potent immunomodulators. Upon activation, they rapidly release preformed granule‐associated mediators including histamine and lipid mediators such as LTC4, while cytokine production occurs over a longer timescale, contributing to downstream immune responses.Jitesh Chauhan, Sofia Vlasova, Alicia Chenoweth, Debra H. Josephs, Heather J. Bax, Sophia N. Karagiannis +5 morewiley +1 more source