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1. International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Author

Farkas, H., Martinez‐Saguer, I., Bork, K., Bowen, T., Craig, T., Frank, M., Germenis, A. E., Grumach, A. S., Luczay, A., Varga, L., Zanichelli, A., Aberer, Werner, Andrejevic, Sladjana, Aygoeren‐Pürsün, Emel, Banerji, Alena, Bara, Noemi‐Anna, Bas, Murat, Bernstein, Jonathan, Betschel, Stephen, Björkander, Janne, Boccon‐Gibod, Isabelle, Bouillet, Laurence, Bova, Maria, Boysen, Henrik Halle, Branco‐Ferreira, Manuel, Bygum, Anette, Caballero, Teresa, Cancian, Mauro, Castaldo, Anthony, Christiansen, Sandra, Cicardi, Marco, Drouet, Christian, Fabiani, Jose, Gompels, Mark, Gonzalez‐Quevedo, Maria Teresa, Gooi, Jimmy, Gower, Richard, Gökmen, Nihal Mete, Grivcheva‐Panovska, Vesna, Guilarte, Mar, Gülbahar, Okan, Hack, Erik, Hakl, Roman, Harmat, György, Jeseňák, Miloš, Jolles, Stephen, Kaplan, Allen, Katelaris, Connie, Kosnik, Mitja, Kőhalmi, Kinga Viktória, Leibovich, Iris, Levi, Marcel, Li, Henry, Longhurst, Hilary J., Lumry, William, Magerl, Markus, Malbran, Alejandro, Martin, Ludovic, Maurer, Marcus, Mihály, Enikő, Moldovan, Dumitru, Murdjeva, Mariana, Nagy, Imola Beatrix, Nielsen, Erik W., Nieto, Sandra, Nordenfelt, Patrik, Obtulowitzc, Kristine, Pedrosa, Maria, Porębski, Grzegorz, Prior, Nieves, Reshef, Avner, Riedl, Marc A., Rosenkranz, Bernd, Schmid‐Grendelmeier, Peter, Péter, Spath, Speletas, Matthaios, Staevska, Maria, Stobiecki, Marcin, Triggiani, Massimo, Veszeli, Nóra, Wuillemin, Walter, Xiang, Zhi Yu, Yamamoto, Beverley, and Zuraw, Bruce

Published
2017
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2. The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

Author

Maurer, Marcus, primary, Magerl, Markus, additional, Betschel, Stephen, additional, Aberer, Werner, additional, Ansotegui, Ignacio J., additional, Aygören‐Pürsün, Emel, additional, Banerji, Aleena, additional, Bara, Noémi‐Anna, additional, Boccon‐Gibod, Isabelle, additional, Bork, Konrad, additional, Bouillet, Laurence, additional, Boysen, Henrik Balle, additional, Brodszki, Nicholas, additional, Busse, Paula J., additional, Bygum, Anette, additional, Caballero, Teresa, additional, Cancian, Mauro, additional, Castaldo, Anthony, additional, Cohn, Danny M., additional, Csuka, Dorottya, additional, Farkas, Henriette, additional, Gompels, Mark, additional, Gower, Richard, additional, Grumach, Anete S., additional, Guidos‐Fogelbach, Guillermo, additional, Hide, Michihiro, additional, Kang, Hye‐Ryun, additional, Kaplan, Allen Phillip, additional, Katelaris, Constance, additional, Kiani‐Alikhan, Sorena, additional, Lei, Wei‐Te, additional, Lockey, Richard, additional, Longhurst, Hilary, additional, Lumry, William R., additional, MacGinnitie, Andrew, additional, Malbran, Alejandro, additional, Martinez Saguer, Inmaculada, additional, Matta, Juan José, additional, Nast, Alexander, additional, Nguyen, Dinh, additional, Nieto‐Martinez, Sandra A., additional, Pawankar, Ruby, additional, Peter, Jonathan, additional, Porebski, Grzegorz, additional, Prior, Nieves, additional, Reshef, Avner, additional, Riedl, Marc, additional, Ritchie, Bruce, additional, Rafique Sheikh, Farrukh, additional, Smith, William B., additional, Spaeth, Peter J., additional, Stobiecki, Marcin, additional, Toubi, Elias, additional, Varga, Lilian Agnes, additional, Weller, Karsten, additional, Zanichelli, Andrea, additional, Zhi, Yuxiang, additional, Zuraw, Bruce, additional, and Craig, Timothy, additional

Published
2022
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3. Long-term safety of icatibant treatment of patients with angioedema in real-world clinical practice

Author

Zanichelli, A., Maurer, M., Aberer, W., Caballero, T., Longhurst, H. J., Bouillet, L., Fabien, V., Andresen, I., Grumach, A., Bygum, A., Blanchard Delaunay, C., Boccon-Gibod, I., Coppere, B., Du Thanh, A., Dzviga, C., Fain, O., Goichot, B., Gompel, A., Guez, S., Jeandel, P., Kanny, G., Launay, D., Maillard, H., Martin, L., Masseau, A., Ollivier, Y., Sobel, A., Aygören-Pürsün, E., Bas, M., Bauer, A., Bork, K., Greve, J., Magerl, M., Martinez Saguer, I., Strassen, U., Papadopoulou-Alataki, E., Psarros, F., Graif, Y., Kivity, S., Reshef, A., Toubi, E., Arcoleo, F., Bova, M., Cicardi, M., Manconi, P., Marone, G., Montinaro, V., Triggiani, M., Baeza, L., Cabañas, R., Gala Ortiz, G., Guilarte, M., Hernandez, D., Hernando de Larramendi, C., Lleonart, R., Lobera, T., Marques, L., Saenz de San Pedro, B., Björkander, J., Bethune, C., Garcez, T., Zanichelli, A., Maurer, M., Aberer, W., Caballero, T., Longhurst, H. J., Bouillet, L., Fabien, V., Andresen, I., Grumach, A., Bygum, A., Blanchard Delaunay, C., Boccon-Gibod, I., Coppere, B., Du Thanh, A., Dzviga, C., Fain, O., Goichot, B., Gompel, A., Guez, S., Jeandel, P., Kanny, G., Launay, D., Maillard, H., Martin, L., Masseau, A., Ollivier, Y., Sobel, A., Aygören-Pürsün, E., Bas, M., Bauer, A., Bork, K., Greve, J., Magerl, M., Martinez Saguer, I., Strassen, U., Papadopoulou-Alataki, E., Psarros, F., Graif, Y., Kivity, S., Reshef, A., Toubi, E., Arcoleo, F., Bova, M., Cicardi, M., Manconi, P., Marone, G., Montinaro, V., Triggiani, M., Baeza, L., Cabañas, R., Gala Ortiz, G., Guilarte, M., Hernandez, D., Hernando de Larramendi, C., Lleonart, R., Lobera, T., Marques, L., Saenz de San Pedro, B., Björkander, J., Bethune, C., and Garcez, T.

Subjects
safety, 0301 basic medicine, real-world, medicine.medical_specialty, Immunology, Brief Communication, Off-label use, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Icatibant, Internal medicine, angioedema, icatibant, Immunology and Allergy, Medicine, Reflux esophagitis, Adverse effect, Pregnancy, Angioedema, business.industry, real‐world, medicine.disease, 030104 developmental biology, 030228 respiratory system, chemistry, Anesthesia, Observational study, Long term safety, medicine.symptom, business
Abstract

The Icatibant Outcome Survey (IOS) is an observational study monitoring safety and effectiveness of icatibant in the real‐world setting. We analyzed safety data from 3025 icatibant‐treated attacks in 557 patients (enrolled between July 2009 and February 2015). Icatibant was generally well tolerated. Excluding off‐label use and pregnancy, 438 patients (78.6%) did not report adverse events (AEs). The remaining 119 (21.4%) patients reported 341 AEs, primarily gastrointestinal disorders (19.6%). Of these, 43 AEs in 17 patients (3.1%) were related to icatibant. Serious AEs (SAEs) occurred infrequently. A total of 143 SAEs occurred in 59 (10.6%) patients; only three events (drug inefficacy, gastritis, and reflux esophagitis) in two patients were considered related to icatibant. Notably, no SAEs related to icatibant occurred in patients with cardiovascular disease, nor in those using icatibant at a frequency above label guidelines. Additionally, no major differences were noted in AEs occurring in on‐label vs off‐label icatibant users.

Published
2017
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4. Definition, aims, and implementation of GA

Author

Marcus, Maurer, Werner, Aberer, Rosana, Agondi, Mona, Al-Ahmad, Maryam Ali, Al-Nesf, Ignacio, Ansotegui, Rand, Arnaout, Luisa Karla, Arruda, Riccardo, Asero, Emel, Aygören-Pürsün, Aleena, Banerji, Andrea, Bauer, Moshe, Ben-Shoshan, Alejandro, Berardi, Jonathan A, Bernstein, Stephen, Betschel, Carsten, Bindslev-Jensen, Mojca, Bizjak, Isabelle, Boccon-Gibod, Konrad, Bork, Laurence, Bouillet, Henrik Balle, Boysen, Nicholas, Brodszki, Sigurd, Broesby-Olsen, Paula, Busse, Thomas, Buttgereit, Anette, Bygum, Teresa, Caballero, Régis A, Campos, Mauro, Cancian, Ivan, Cherrez-Ojeda, Danny M, Cohn, Célia, Costa, Timothy, Craig, Paulo Ricardo, Criado, Roberta F, Criado, Dorottya, Csuka, Joachim, Dissemond, Aurélie, Du-Thanh, Luis Felipe, Ensina, Ragıp, Ertaş, José E, Fabiani, Claudio, Fantini, Henriette, Farkas, Silvia Mariel, Ferrucci, Ignasi, Figueras-Nart, Natalia L, Fili, Daria, Fomina, Atsushi, Fukunaga, Asli, Gelincik, Ana, Giménez-Arnau, Kiran, Godse, Mark, Gompels, Margarida, Gonçalo, Maia, Gotua, Richard, Gower, Anete S, Grumach, Guillermo, Guidos-Fogelbach, Michihiro, Hide, Natalia, Ilina, Naoko, Inomata, Thilo, Jakob, Dario O, Josviack, Hye-Ryun, Kang, Allen, Kaplan, Alicja, Kasperska-Zając, Constance, Katelaris, Aharon, Kessel, Andreas, Kleinheinz, Emek, Kocatürk, Mitja, Košnik, Dorota, Krasowska, Kanokvalai, Kulthanan, M Sendhil, Kumaran, José Ignacio, Larco Sousa, Hilary J, Longhurst, William, Lumry, Andrew, MacGinnitie, Markus, Magerl, Michael P, Makris, Alejandro, Malbrán, Alexander, Marsland, Inmaculada, Martinez-Saguer, Iris V, Medina, Raisa, Meshkova, Martin, Metz, Iman, Nasr, Jan, Nicolay, Chikako, Nishigori, Isao, Ohsawa, Kemal, Özyurt, Nikolaos G, Papadopoulos, Claudio A S, Parisi, Jonathan Grant, Peter, Wolfgang, Pfützner, Todor, Popov, Nieves, Prior, German D, Ramon, Adam, Reich, Avner, Reshef, Marc A, Riedl, Bruce, Ritchie, Heike, Röckmann-Helmbach, Michael, Rudenko, Andaç, Salman, Mario, Sanchez-Borges, Peter, Schmid-Grendelmeier, Faradiba S, Serpa, Esther, Serra-Baldrich, Farrukh R, Sheikh, William, Smith, Angèle, Soria, Petra, Staubach, Urs C, Steiner, Marcin, Stobiecki, Gordon, Sussman, Anna, Tagka, Simon Francis, Thomsen, Regina, Treudler, Solange, Valle, Martijn, van Doorn, Lilian, Varga, Daniel O, Vázquez, Nicola, Wagner, Liangchun, Wang, Christina, Weber-Chrysochoou, Young-Min, Ye, Anna, Zalewska-Janowska, Andrea, Zanichelli, Zuotao, Zhao, Yuxiang, Zhi, Torsten, Zuberbier, Ricardo D, Zwiener, and Anthony, Castaldo

Subjects
Urticaria, Humans, Angioedema
Published
2020

5. Definition, aims, and implementation of GA2LEN/HAEi Angioedema Centers of Reference and Excellence

Author

Maurer, M. (Marcus), Aberer, W. (W.), Agondi, R. (Rosana), Al-Ahmad, M. (Mona), Al-Nesf, M.A. (Maryam Ali), Ansotegui, I.J. (I.), Arnaout, R. (Rand), Arruda, L.K. (Luisa Karla), Asero, R. (Riccardo), Aygören-Pürsü, E. (Emel), Banerji, A. (Aleena), Bauer, A. (Andrea), Ben-Shoshan, M. (Moshe), Berardi, A. (Alejandro), Bernstein, J.A. (Jonathan A.), Betschel, S. (Stephen), Bindslev-Jensen, C. (Carsten), Bizjak, M. (Mojca), Boccon-Gibod, I. (Isabelle), Bork, K. (Konrad), Bouillet, L. (Laurence), Boysen, H.B. (Henrik Balle), Brodszki, N. (N.), Broesby-Olsen, S. (Sigurd), Busse, P. (Paula), Buttgereit, T. (Thomas), Bygum, A. (Anette), Caballero, T. (Teresa), Campos, R.A. (Régis A.), Cancian, M. (Mauro), Cherrez-Ojeda, I. (Ivan), Cohn, D.M. (Danny M.), Costa, C. (Célia), Craig, T., Criado, P.R. (Paulo Ricardo), Criado, R.F. (Roberta F.), Csuka, D. (Dorottya), Dissemond, J. (Joachim), Du-Thanh, A. (Aurélie), Ensina, L.F. (Luis Felipe), Ertaş, R. (Ragıp), Fabiani, J.E. (José E.), Fantini, C. (Claudio), Farkas, H. (Henriette), Ferrucci, S.M. (Silvia Mariel), Figueras-Nart, I. (Ignasi), Fili, N.L. (Natalia L.), Fomina, D. (Daria), Fukunaga, A. (Atsushi), Gelincik, A. (Asli), Giménez-Arnau, A., Godse, K., Gompels, M. (Mark), Gonçalo, M. (Margarida), Gotua, M. (M.), Gower, R. (Richard), Grumach, A.S. (Anete S.), Guidos-Fogelbach, G. (Guillermo), Hide, M. (Michihiro), Ilina, N. (Natalia), Inomata, N. (Naoko), Jakob, T. (Thilo), Josviack, D.O. (Dario O.), Kang, H.-R. (Hye-Ryun), Kaplan, A. (Allen), Kasperska-Zając, A. (Alicja), Katelaris, C. (Constance), Kessel, A. (Aharon), Kleinheinz, A. (Andreas), Kocatürk, E., Košnik, M. (Mitja), Krasowska, D. (Dorota), Kulthanan, K. (Kanokvalai), Kumaran, M.S. (M. Sendhil), Larco Sousa, J.I. (José Ignacio), Longhurst, H.J. (Hilary J.), Lumry, W. (William), MacGinnitie, A. (Andrew), Magerl, M. (Markus), Makris, M.P. (Michael P.), Malbrán, A. (Alejandro), Marsland, A. (Alexander), Martinez-Saguer, I. (Inmaculada), Medina, I.V. (Iris V.), Meshkova, R. (Raisa), Metz, M. (Martin), Nasr, I. (Iman), Nicolay, J. (Jan), Nishigori, C. (Chikako), Ohsawa, I. (Isao), Özyurt, K. (Kemal), Papadopoulos, N., Parisi, C.A.S. (Claudio A. S.), Peter, J.G. (Jonathan Grant), Pfützner, W. (W.), Popov, T.A., Prior, N. (Nieves), Ramon, G.D. (German D.), Reich, A. (Adam), Reshef, A. (Avner), Riedl, M.A. (Marc A.), Ritchie, B. (Bruce), Röckmann-Helmbach, H. (Heike), Rudenko, M. (Michael), Salman, A. (Andaç), Sanchez-Borges, M. (Mario), Schmid-Grendelmeier, P. (Peter), Serpa, F.S. (Faradiba S.), Serra-Baldrich, E. (Esther), Sheikh, F.R. (Farrukh R.), Smith, W. (William), Soria, A. (Angèle), Staubach, P. (Petra), Steiner, U.C. (Urs C.), Stobiecki, M. (Marcin), Sussman, G. (Gordon), Tagka, A. (Anna), Thomsen, S.F. (Simon Francis), Treudler, R. (Regina), Valle, S. (Solange), Doorn, M.B.A. (Martijn) van, Varga, L. (Lilian), Vázquez, D.O. (Daniel O.), Wagner, N. (Nicola), Wang, L. (Liangchun), Weber-Chrysochoou, C. (Christina), Ye, Y.-M. (Young-Min), Zalewska-Janowska, A. (Anna), Zanichelli, A. (Andrea), Zhao, Z. (Zuotao), Zhi, Y. (Yuxiang), Zuberbier, T. (Torsten), Zwiener, R.D. (Ricardo D.), Castaldo, A. (Anthony), Maurer, M. (Marcus), Aberer, W. (W.), Agondi, R. (Rosana), Al-Ahmad, M. (Mona), Al-Nesf, M.A. (Maryam Ali), Ansotegui, I.J. (I.), Arnaout, R. (Rand), Arruda, L.K. (Luisa Karla), Asero, R. (Riccardo), Aygören-Pürsü, E. (Emel), Banerji, A. (Aleena), Bauer, A. (Andrea), Ben-Shoshan, M. (Moshe), Berardi, A. (Alejandro), Bernstein, J.A. (Jonathan A.), Betschel, S. (Stephen), Bindslev-Jensen, C. (Carsten), Bizjak, M. (Mojca), Boccon-Gibod, I. (Isabelle), Bork, K. (Konrad), Bouillet, L. (Laurence), Boysen, H.B. (Henrik Balle), Brodszki, N. (N.), Broesby-Olsen, S. (Sigurd), Busse, P. (Paula), Buttgereit, T. (Thomas), Bygum, A. (Anette), Caballero, T. (Teresa), Campos, R.A. (Régis A.), Cancian, M. (Mauro), Cherrez-Ojeda, I. (Ivan), Cohn, D.M. (Danny M.), Costa, C. (Célia), Craig, T., Criado, P.R. (Paulo Ricardo), Criado, R.F. (Roberta F.), Csuka, D. (Dorottya), Dissemond, J. (Joachim), Du-Thanh, A. (Aurélie), Ensina, L.F. (Luis Felipe), Ertaş, R. (Ragıp), Fabiani, J.E. (José E.), Fantini, C. (Claudio), Farkas, H. (Henriette), Ferrucci, S.M. (Silvia Mariel), Figueras-Nart, I. (Ignasi), Fili, N.L. (Natalia L.), Fomina, D. (Daria), Fukunaga, A. (Atsushi), Gelincik, A. (Asli), Giménez-Arnau, A., Godse, K., Gompels, M. (Mark), Gonçalo, M. (Margarida), Gotua, M. (M.), Gower, R. (Richard), Grumach, A.S. (Anete S.), Guidos-Fogelbach, G. (Guillermo), Hide, M. (Michihiro), Ilina, N. (Natalia), Inomata, N. (Naoko), Jakob, T. (Thilo), Josviack, D.O. (Dario O.), Kang, H.-R. (Hye-Ryun), Kaplan, A. (Allen), Kasperska-Zając, A. (Alicja), Katelaris, C. (Constance), Kessel, A. (Aharon), Kleinheinz, A. (Andreas), Kocatürk, E., Košnik, M. (Mitja), Krasowska, D. (Dorota), Kulthanan, K. (Kanokvalai), Kumaran, M.S. (M. Sendhil), Larco Sousa, J.I. (José Ignacio), Longhurst, H.J. (Hilary J.), Lumry, W. (William), MacGinnitie, A. (Andrew), Magerl, M. (Markus), Makris, M.P. (Michael P.), Malbrán, A. (Alejandro), Marsland, A. (Alexander), Martinez-Saguer, I. (Inmaculada), Medina, I.V. (Iris V.), Meshkova, R. (Raisa), Metz, M. (Martin), Nasr, I. (Iman), Nicolay, J. (Jan), Nishigori, C. (Chikako), Ohsawa, I. (Isao), Özyurt, K. (Kemal), Papadopoulos, N., Parisi, C.A.S. (Claudio A. S.), Peter, J.G. (Jonathan Grant), Pfützner, W. (W.), Popov, T.A., Prior, N. (Nieves), Ramon, G.D. (German D.), Reich, A. (Adam), Reshef, A. (Avner), Riedl, M.A. (Marc A.), Ritchie, B. (Bruce), Röckmann-Helmbach, H. (Heike), Rudenko, M. (Michael), Salman, A. (Andaç), Sanchez-Borges, M. (Mario), Schmid-Grendelmeier, P. (Peter), Serpa, F.S. (Faradiba S.), Serra-Baldrich, E. (Esther), Sheikh, F.R. (Farrukh R.), Smith, W. (William), Soria, A. (Angèle), Staubach, P. (Petra), Steiner, U.C. (Urs C.), Stobiecki, M. (Marcin), Sussman, G. (Gordon), Tagka, A. (Anna), Thomsen, S.F. (Simon Francis), Treudler, R. (Regina), Valle, S. (Solange), Doorn, M.B.A. (Martijn) van, Varga, L. (Lilian), Vázquez, D.O. (Daniel O.), Wagner, N. (Nicola), Wang, L. (Liangchun), Weber-Chrysochoou, C. (Christina), Ye, Y.-M. (Young-Min), Zalewska-Janowska, A. (Anna), Zanichelli, A. (Andrea), Zhao, Z. (Zuotao), Zhi, Y. (Yuxiang), Zuberbier, T. (Torsten), Zwiener, R.D. (Ricardo D.), and Castaldo, A. (Anthony)

Published
2020
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6. Definition, aims, and implementation of GA 2 LEN/HAEi Angioedema Centers of Reference and Excellence

Author

Maurer, Marcus, primary, Aberer, Werner, additional, Agondi, Rosana, additional, Al‐Ahmad, Mona, additional, Al‐Nesf, Maryam Ali, additional, Ansotegui, Ignacio, additional, Arnaout, Rand, additional, Arruda, Luisa Karla, additional, Asero, Riccardo, additional, Aygören‐Pürsün, Emel, additional, Banerji, Aleena, additional, Bauer, Andrea, additional, Ben‐Shoshan, Moshe, additional, Berardi, Alejandro, additional, Bernstein, Jonathan A., additional, Betschel, Stephen, additional, Bindslev‐Jensen, Carsten, additional, Bizjak, Mojca, additional, Boccon‐Gibod, Isabelle, additional, Bork, Konrad, additional, Bouillet, Laurence, additional, Boysen, Henrik Balle, additional, Brodszki, Nicholas, additional, Broesby‐Olsen, Sigurd, additional, Busse, Paula, additional, Buttgereit, Thomas, additional, Bygum, Anette, additional, Caballero, Teresa, additional, Campos, Régis A., additional, Cancian, Mauro, additional, Cherrez‐Ojeda, Ivan, additional, Cohn, Danny M., additional, Costa, Célia, additional, Craig, Timothy, additional, Criado, Paulo Ricardo, additional, Criado, Roberta F., additional, Csuka, Dorottya, additional, Dissemond, Joachim, additional, Du‐Thanh, Aurélie, additional, Ensina, Luis Felipe, additional, Ertaş, Ragıp, additional, Fabiani, José E., additional, Fantini, Claudio, additional, Farkas, Henriette, additional, Ferrucci, Silvia Mariel, additional, Figueras‐Nart, Ignasi, additional, Fili, Natalia L., additional, Fomina, Daria, additional, Fukunaga, Atsushi, additional, Gelincik, Asli, additional, Giménez‐Arnau, Ana, additional, Godse, Kiran, additional, Gompels, Mark, additional, Gonçalo, Margarida, additional, Gotua, Maia, additional, Gower, Richard, additional, Grumach, Anete S., additional, Guidos‐Fogelbach, Guillermo, additional, Hide, Michihiro, additional, Ilina, Natalia, additional, Inomata, Naoko, additional, Jakob, Thilo, additional, Josviack, Dario O., additional, Kang, Hye‐Ryun, additional, Kaplan, Allen, additional, Kasperska‐Zając, Alicja, additional, Katelaris, Constance, additional, Kessel, Aharon, additional, Kleinheinz, Andreas, additional, Kocatürk, Emek, additional, Košnik, Mitja, additional, Krasowska, Dorota, additional, Kulthanan, Kanokvalai, additional, Kumaran, M. Sendhil, additional, Larco Sousa, José Ignacio, additional, Longhurst, Hilary J., additional, Lumry, William, additional, MacGinnitie, Andrew, additional, Magerl, Markus, additional, Makris, Michael P., additional, Malbrán, Alejandro, additional, Marsland, Alexander, additional, Martinez‐Saguer, Inmaculada, additional, Medina, Iris V., additional, Meshkova, Raisa, additional, Metz, Martin, additional, Nasr, Iman, additional, Nicolay, Jan, additional, Nishigori, Chikako, additional, Ohsawa, Isao, additional, Özyurt, Kemal, additional, Papadopoulos, Nikolaos G., additional, Parisi, Claudio A. S., additional, Peter, Jonathan Grant, additional, Pfützner, Wolfgang, additional, Popov, Todor, additional, Prior, Nieves, additional, Ramon, German D., additional, Reich, Adam, additional, Reshef, Avner, additional, Riedl, Marc A., additional, Ritchie, Bruce, additional, Röckmann‐Helmbach, Heike, additional, Rudenko, Michael, additional, Salman, Andaç, additional, Sanchez‐Borges, Mario, additional, Schmid‐Grendelmeier, Peter, additional, Serpa, Faradiba S., additional, Serra‐Baldrich, Esther, additional, Sheikh, Farrukh R., additional, Smith, William, additional, Soria, Angèle, additional, Staubach, Petra, additional, Steiner, Urs C., additional, Stobiecki, Marcin, additional, Sussman, Gordon, additional, Tagka, Anna, additional, Thomsen, Simon Francis, additional, Treudler, Regina, additional, Valle, Solange, additional, van Doorn, Martijn, additional, Varga, Lilian, additional, Vázquez, Daniel O., additional, Wagner, Nicola, additional, Wang, Liangchun, additional, Weber‐Chrysochoou, Christina, additional, Ye, Young‐Min, additional, Zalewska‐Janowska, Anna, additional, Zanichelli, Andrea, additional, Zhao, Zuotao, additional, Zhi, Yuxiang, additional, Zuberbier, Torsten, additional, Zwiener, Ricardo D., additional, and Castaldo, Anthony, additional

Published
2020
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7. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2017 revision and update

Author

Maurer, M., primary, Magerl, M., additional, Ansotegui, I., additional, Aygören-Pürsün, E., additional, Betschel, S., additional, Bork, K., additional, Bowen, T., additional, Balle Boysen, H., additional, Farkas, H., additional, Grumach, A. S., additional, Hide, M., additional, Katelaris, C., additional, Lockey, R., additional, Longhurst, H., additional, Lumry, W. R., additional, Martinez-Saguer, I., additional, Moldovan, D., additional, Nast, A., additional, Pawankar, R., additional, Potter, P., additional, Riedl, M., additional, Ritchie, B., additional, Rosenwasser, L., additional, Sánchez-Borges, M., additional, Zhi, Y., additional, Zuraw, B., additional, and Craig, T., additional

Published
2018
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8. Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group

Author

Roberto Perricone, Anette Bygum, Caterina De Carolis, Vincenzo Montinaro, Richard Gower, Marco Cicardi, Alejandro Malbran, Avner Reshef, Anete Grumach, Konrad Bork, Mar Guilarte, Lorenza Chiara Zingale, Henriette Farkas, Andrea Zanichelli, MASSIMO TRIGGIANI, Teresa Caballero, Marcus Maurer, Bruce Zuraw, and Lilian Varga

Subjects
medicine.medical_specialty, Evidence-based practice, Angioedema, biology, business.industry, Immunology, Alternative medicine, Lanadelumab, medicine.disease, C1-inhibitor, Ecallantide, Family medicine, Hereditary angioedema, medicine, biology.protein, Immunology and Allergy, Observational study, medicine.symptom, business, medicine.drug
Abstract

Angioedema owing to hereditary deficiency of C1 inhibitor (HAE) is a rare, life-threatening, disabling disease. In the last 2 years, the results of well-designed and controlled trials with existing and new therapies for this condition have been published, and new treatments reached the market. Current guidelines for the treatment for HAE were released before the new trials and before the new treatments became available and were essentially based on observational studies and expert opinion. To provide evidence-based HAE treatment guidelines supported by the new studies, a conference was held in Gargnano del Garda, Italy, from September 26 to 29, 2010. The meeting hosted 58 experienced HAE expert physicians, representatives of pharmaceutical companies and representatives of HAE patients' associations. Here, we report the topics discussed during the meeting and evidence-based consensus about management approaches for HAE in adult/adolescent patients.

Published
2011
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9. Homozygosity for a factor XII mutation in one female and one male patient with hereditary angio-oedema

Author

Anete Sevciovic Grumach, Markus M. Nöthen, Elisabete Cordeiro, Christiane Stieber, João Bosco Pesquero, Sven Cichon, Camila Lopes Veronez, Nathália Cagini, and Rosemeire Navickas Constantino-Silva

Subjects
0301 basic medicine, Adult, Male, medicine.medical_specialty, Immunology, C1-inhibitor, 03 medical and health sciences, 0302 clinical medicine, Mutation Carrier, Internal medicine, medicine, Immunology and Allergy, Humans, Allele, Codon, Gene, Alleles, Aged, Genetics, Factor XII, biology, Homozygote, Angioedemas, Hereditary, Middle Aged, Phenotype, Pedigree, 030104 developmental biology, Endocrinology, 030228 respiratory system, Amino Acid Substitution, Male patient, Mutation (genetic algorithm), Mutation, biology.protein, Female, Brazil
Abstract

Hereditary angio-oedema (HAE) with normal C1 inhibitor is associated with heterozygous mutations in the factor XII gene (FXII-HAE). We report two Brazilian FXII-HAE families segregating the mutation c.983 C>A (p.Thr328Lys). In each family, one patient with a homozygous mutation was found. The homozygous female patient in family 1 displayed a severe phenotype. However, this falls within the clinical phenotype spectrum reported for heterozygous female mutation carriers. The homozygous male patient in family 2 also showed a severe phenotype. This finding is intriguing, as to our knowledge, it is the first such report for a male FXII-HAE mutation carrier. In the rare instances in which male mutation carriers are affected, a mild phenotype is typical. The present findings therefore suggest that homozygous FXII-HAE mutation status leads to a severe phenotype in females and males, and to an increased risk of manifest symptoms in the latter.

Published
2015

11. Homozygosity for a factor XII mutation in one female and one male patient with hereditary angio-oedema.

Author

Grumach, A. S., Stieber, C., Veronez, C. L., Cagini, N., Constantino‐Silva, R. N., Cordeiro, E., Nöthen, M. M., Pesquero, J. B., and Cichon, S.

Subjects
*HOMOZYGOSITY, *WOMEN patients
Abstract

Hereditary angio‐oedema (HAE) with normal C1 inhibitor is associated with heterozygous mutations in the factor XII gene (FXII‐HAE). We report two Brazilian FXII‐HAE families segregating the mutation c.983 C>A (p.Thr328Lys). In each family, one patient with a homozygous mutation was found. The homozygous female patient in family 1 displayed a severe phenotype. However, this falls within the clinical phenotype spectrum reported for heterozygous female mutation carriers. The homozygous male patient in family 2 also showed a severe phenotype. This finding is intriguing, as to our knowledge, it is the first such report for a male FXII‐HAE mutation carrier. In the rare instances in which male mutation carriers are affected, a mild phenotype is typical. The present findings therefore suggest that homozygous FXII‐HAE mutation status leads to a severe phenotype in females and males, and to an increased risk of manifest symptoms in the latter. [ABSTRACT FROM AUTHOR]

Published
2016
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12. Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group.

Author

Cicardi, M., Aberer, W., Banerji, A., Bas, M., Bernstein, J. A., Bork, K., Caballero, T., Farkas, H., Grumach, A., Kaplan, A. P., Riedl, M. A., Triggiani, M., Zanichelli, A., and Zuraw, B.

Subjects
ANGIONEUROTIC edema, REPORTING of diseases, VASOCONSTRICTORS, CLINICAL immunology, MEDICAL databases, DIAGNOSIS, THERAPEUTICS
Abstract

Angioedema is defined as localized and self-limiting edema of the subcutaneous and submucosal tissue, due to a temporary increase in vascular permeability caused by the release of vasoactive mediator(s). When angioedema recurs without significant wheals, the patient should be diagnosed to have angioedema as a distinct disease. In the absence of accepted classification, different types of angioedema are not uniquely identified. For this reason, the European Academy of Allergy and Clinical Immunology gave its patronage to a consensus conference aimed at classifying angioedema. Four types of acquired and three types of hereditary angioedema were identified as separate forms from the analysis of the literature and were presented in detail at the meeting. Here, we summarize the analysis of the data and the resulting classification of angioedema. [ABSTRACT FROM AUTHOR]

Published
2014
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