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Adler, Clemens ; Ahammed, Zubayer ; Allgower, Chris ; Amonett, John ; Anderson, Bryon D. ; Anderson, Mike ; Averichev, Georgy S. ; Balewski, Jan ; Barannikova, Olga ; Barnby, Lee Stuart ; Baudot, Jérome ; Bekele, Selemon ; Belaga, Victoria Vladimirovna ; Bellwied, Rene ; Berger, Jens ; Bichsel, Hans ; Bland, Leslie C. ; Blyth, Charles O. ; Bonner, Billy E. ; Boucham, Abdelkrim ; Brandin, Andrei V. ; Cadman, R. V. ; Caines, Helen Louise ; Calderón de la Barca Sánchez, Manuel ; Cardenas, Andrés ; Carroll, J. ; Castillo Castellanos, Javier Ernesto ; Castro, M. ; Cebra, Daniel A. ; Chattopadhyay, S. ; Chen, M. L. ; Chen, Y. ; Chernenko, Sergey P. ; Cherney, Michael Gerard ; Chikanian, Alexei ; Choi, B. ; Christie, William ; Coffin, Jean Pierre ; Cormier, Thomas Michael ; Cramer, John G. ; Crawford, Henry J. ; DeMello, M. ; Deng, W. S. ; Derevschikov, Anatoly A. ; Didenko, Lidia ; Draper, James E. ; Dunin, V. B. ; Dunlop, James C. ; Eckardt, Volker ; Efimov, Leonid G. ; Emelianov, V. ; Engelage, Jon M. ; Eppley, Geary Wareham ; Erazmus, Barbara Ewa ; Fachini, Patricia ; Faine, V. ; Filimonov, Kirill ; Finch, Evan ; Fisyak, Yuri V. ; Flierl, Dominik Bernhard ; Foley, K. J. ; Fu, J. ; Gagliardi, Carl A. ; Gagunashvili, N. ; Gans, J. ; Gaudichet, L. ; Germain, Marie ; Geurts, Frank ; Ghazikhanian, Vahe ; Grabski, J. ; Grachov, Oleg A. ; Grigoriev, Vladislav A. ; Guedon, M. ; Gushin, E. ; Hallman, Timothy J. ; Hardtke, David H. ; Harris, John W. ; Heffner, Michael D. ; Heppelmann, Steven Francis ; Herston, T. ; Hippolyte, Boris ; Hirsch, Andrew S. ; Hjort, Eric ; Hoffmann, Gerald W. ; Horsley, M. ; Huang, Huan Zhong ; Humanic, Thomas J. ; Hümmler, Holm Gero ; Igo, George J. ; Ishihara, A. ; Ivanshin, Yu. I. ; Jacobs, Peter Martin ; Jacobs, William W. ; Janik, Michał ; Johnson, I. ; Jones, Peter Graham ; Judd, Eleanor G. ; Kaneta, Kaneta ; Kaplan, Morton ; Keane, Declan ; Kisiel, Adam Ryszard ; Klay, Jennifer Lynn ; Klein, Spencer Robert ; Klyachko, A. ; Konstantinov, A. S. ; Kotchenda, L. ; Kovalenko, A. D. ; Kramer, M. ; Kravtsov, Peter ; Krueger, Keith William ; Kuhn, Christian Claude ; Kulikov, Anatoly I. ; Kunde, Gerd J. ; Kunz, Christopher Lee ; Kutuev, R. Kh. ; Kuznetsov, A. A. ; Lakehal-Ayat, L. ; Lamas-Valverde, Jose ; Lamont, Matthew A. C. ; Landgraf, Jeffery M. ; Lange, S. ; Lansdell, Curtis Patrick Leon ; Lasiuk, Brian ; Laue, Frank ; Lebedev, Alexei ; Lednický, Richard ; Leontiev, V. M. ; LeVine, Micheal J. ; Li, Q. ; Lindenbaum, Sam J. ; Lisa, Michael A. ; Liu, F. ; Liu, L. ; Liu, Z. ; Liu, Q. J. ; Ljubicic, Tonko A. ; Llope, William Joseph ; LoCurto, G. ; Long, H. ; Longacre, Ronald S. ; Lopez-Noriega, M. ; Love, William A. ; Lynn, David ; Majka, Richard Daniel ; Margetis, Spyridon ; Martin, L. ; Marx, Jay N. ; Matis, Howard S. ; Matulenko, Yuri A. ; McShane, Tom S. ; Meissner, F. ; Melnick, Yu. ; Meščanin, Aleksei P. ; Messer, Matthias ; Miller, M. L. ; Milosevich, Z. ; Minaev, Nikolai G. ; Mitchell, J. ; Moiseenko, V. A. ; Moore, C. F. ; Morozov, V. ; de Moura, M. M. ; Munhoz, Marcelo Gameiro ; Mutchler, G. S. ; Nelson, John M. ; Nevski, P. ; Nikitin, Vladimir A. ; Nogach, Larisa V. ; Norman, B. ; Nurušev, Sandibek B. ; Odyniec, Grazyna Janina ; Ogawa, Akio ; Okorokov, Vitaly A. ; Oldenburg, Markus D. ; Olson, Douglas L. ; Paic, Guy ; Pandey, S. U. ; Panebratsev, Yuri ; Panitkin, Sergei Yurievich ; Pavlinov, A. I. ; Pawlak, Tomasz Jan ; Perevoztchikov, V. ; Peryt, Wiktor Stanislaw ; Petrov, V. A. ; Platner, E. ; Pluta, Jan Marian ; Porile, Norbert T. ; Porter, Jeffrey Brent ; Poskanzer, Arthur M. ; Potrebenikova, E. ; Prindle, Duncan J. ; Pruneau, Claude A. ; Radomski, S. ; Rai, Gulshan ; Ravel, Olivier ; Ray, Robert L. ; Razin, Sergej V. ; Reichhold, Dennis Michael ; Reid, Jeffrey G. ; Retiere, Fabrice ; Ridiger, Alexey ; Ritter, Hans Georg ; Roberts, Jabus B. ; Rogachevski, O. V. ; Romero, Juan L. ; Roy, Christelle Sophie ; Rykov, Vladimir L. ; Sakrejda, Iwona ; Sandweiss, Jack H. ; Saulys, A. C. ; Savin, Igor A. ; Schambach, Joachim ; Scharenberg, Rolf Paul ; Schmitz, Norbert ; Schroeder, Lee S. ; Schüttauf, Andreas ; Schweda, Kai ; Seger, Janet Elizabeth ; Seliverstov, D. ; Seyboth, Peter ; Shahaliev, Ehtiram ; Shestermanov, Konstantin E. ; Shimanskii, S. S. ; Shvetcov, V. S. ; Skoro, Goran P. ; Smirnov, Nikolai ; Snellings, Raimond ; Sowinski, James ; Spinka, Harold. M. ; Srivastava, Brijesh Kumar ; Stephenson, Edward J. ; Stock, Reinhard ; Stolpovsky, Alexander ; Strichanov, Michail Nikolaevič ; Stringfellow, Blair ; Struck, Christof ; Suaide, Alexandre Alarcon do Passo ; Sugarbaker, Evan R. ; Suire, Christophe Pierre ; Šumbera, Michal ; Symons, Timothy James MacNeil ; Toledo, Alejandro Szanto de ; Szarwas, Piotr ; Takahashi, Jun ; Tang, Aihong ; Thomas, James H. ; Thompson, M. ; Tikhomirov, V. ; Trainor, Thomas A. ; Trentalange, Stephen ; Tribble, Robert E. ; Tokarev, Mikhail V. ; Tonjes, Marguerite Belt ; Trofimov, V. ; Tsai, Oleg D. ; Turner, K. ; Ullrich, Thomas ; Underwood, David G. ; Van Buren, Gene ; VanderMolen, A. M. ; Vanyashin, A. ; Vasilevski, I. M. ; Vasiliev, Alexander Nikolaevich ; Vigdor, Steven E. ; Voloshin, Sergei ; Wang, F. ; Ward, H. ; Watson, J. W. ; Wells, R. ; Wenaus, T. ; Westfall, Gary D. ; Whitten, Charles A. ; Wieman, Howard ; Willson, Robert ; Wissink, Scott W. ; Witt, Richard ; Xu, N. ; Xu, Z. ; Yakutin, A. E. ; Yamamoto, E. ; Yang, J. ; Yepes, Pablo P. ; Yurevich, Vladimir Yurevich ; Zanevski, Y. V. ; Zborovský, Imrich ; Zhang, Haitao ; Zhang, W. M. ; Zoulkarneev, R. ; Zubarev, A. N.
We report the first measurement of inclusive antiproton production at midrapidity in Au+Au collisions at sqrt[sNN] = 130 GeV by the STAR experiment at RHIC. The antiproton transverse mass distributions in the measured transverse momentum range of 0.25<pperp<0.95 GeV/c are found to fall less steeply for more central collisions. The extrapolated antiproton rapidity density is found to scale approximately with the negative hadron multiplicity density.
Adler, Clemens ; Ahammed, Zubayer ; Allgower, Chris ; Anderson, Mike ; Averichev, Georgy S. ; Balewski, Jan ; Barannikova, Olga ; Barnby, Lee Stuart ; Baudot, Jérome ; Bekele, Selemon ; Belaga, Victoria Vladimirovna ; Bellwied, Rene ; Berger, Jens ; Bichsel, Hans ; Bland, Leslie C. ; Blyth, Charles O. ; Bonner, Billy E. ; Bossingham, R. ; Boucham, Abdelkrim ; Brandin, Andrei V. ; Caines, Helen Louise ; Calderón de la Barca Sánchez, Manuel ; Cardenas, Andrés ; Carroll, J. ; Castillo Castellanos, Javier Ernesto ; Castro, M. ; Cebra, Daniel A. ; Chattopadhyay, S. ; Chen, M. L. ; Chen, Y. ; Chernenko, Sergey P. ; Cherney, Michael Gerard ; Chikanian, Alexei ; Choi, B. ; Christie, William ; Coffin, Jean Pierre ; Conin, Laurent ; Cormier, Thomas Michael ; Cramer, John G. ; Crawford, Henry J. ; DeMello, M. ; Deng, W. S. ; Derevschikov, Anatoly A. ; Didenko, Lidia ; Draper, James E. ; Dunin, V. B. ; Dunlop, James C. ; Eckardt, Volker ; Efimov, Leonid G. ; Emelianov, V. ; Engelage, Jon M. ; Eppley, Geary Wareham ; Erazmus, Barbara Ewa ; Fachini, Patricia ; Ferguson, M. I. ; Finch, Evan ; Fisyak, Yuri V. ; Flierl, Dominik Bernhard ; Foley, K. J. ; Gagunashvili, N. ; Gans, J. ; Germain, Marie ; Geurts, Frank ; Ghazikhanian, Vahe ; Grabski, J. ; Grachov, Oleg A. ; Greiner, D. ; Grigoriev, Vladislav A. ; Gushin, E. ; Hallman, Timothy J. ; Hardtke, David H. ; Harris, John W. ; Heffner, Michael D. ; Heppelmann, Steven Francis ; Herston, T. ; Hippolyte, Boris ; Hirsch, Andrew S. ; Hjort, Eric ; Hoffmann, Gerald W. ; Horsley, M. ; Huang, Huan Zhong ; Humanic, Thomas J. ; Hümmler, Holm Gero ; Igo, George J. ; Ishihara, A. ; Ivanshin, Yu. I. ; Jacobs, Peter Martin ; Jacobs, William W. ; Janik, Michał ; Johnson, I. ; Jones, Peter Graham ; Judd, Eleanor G. ; Kaneta, Kaneta ; Kaplan, Morton ; Keane, Declan ; Khodinov, A. ; Kisiel, Adam Ryszard ; Klay, Jennifer Lynn ; Klein, Spencer Robert ; Klyachko, A. ; Konstantinov, A. S. ; Kotchenda, L. ; Kovalenko, A. D. ; Kramer, M. ; Kravtsov, Peter ; Krueger, Keith William ; Kuhn, Christian Claude ; Kulikov, Anatoly I. ; Kunde, Gerd J. ; Kunz, Christopher Lee ; Kutuev, R. Kh. ; Kuznetsov, A. A. ; Lamas-Valverde, Jose ; Lamont, Matthew A. C. ; Landgraf, Jeffery M. ; Lange, S. ; Lansdell, Curtis Patrick Leon ; Lasiuk, Brian ; Laue, Frank ; Lebedev, Alexei ; LeCompte, T. ; Leontiev, V. M. ; Leszczynski, P. ; LeVine, Micheal J. ; Li, Q. ; Li, Q. ; Lindenbaum, Sam J. ; Lisa, Michael A. ; Ljubicic, Tonko A. ; Llope, William Joseph ; LoCurto, G. ; Long, H. ; Longacre, Ronald S. ; Lopez-Noriega, M. ; Love, William A. ; Lynn, David ; Madansky, L. ; Majka, Richard Daniel ; Maliszewski, A. ; Margetis, Spyridon ; Martin, L. ; Marx, Jay N. ; Matis, Howard S. ; Matulenko, Yuri A. ; McShane, Tom S. ; Melnick, Yu. ; Meščanin, Aleksei P. ; Milosevich, Z. ; Minaev, Nikolai G. ; Mitchell, J. ; Moiseenko, V. A. ; Moltz, D. ; Moore, C. F. ; Morozov, V. ; de Moura, M. M. ; Munhoz, Marcelo Gameiro ; Mutchler, G. S. ; Nelson, John M. ; Nevski, P. ; Nikitin, Vladimir A. ; Nogach, Larisa V. ; Norman, B. ; Nurušev, Sandibek B. ; Nystrand, Joakim Ingemar ; Odyniec, Grazyna Janina ; Ogawa, Akio ; Ogilvie, C. A. ; Oldenburg, Markus D. ; Olson, Douglas L. ; Paic, Guy ; Pandey, S. U. ; Panebratsev, Yuri ; Panitkin, Sergei Yurievich ; Pavlinov, A. I. ; Pawlak, Tomasz Jan ; Perevoztchikov, V. ; Peryt, Wiktor Stanislaw ; Petrov, V. A. ; Pinganaud, W. ; Platner, E. ; Pluta, Jan Marian ; Porile, Norbert T. ; Porter, Jeffrey Brent ; Poskanzer, Arthur M. ; Potrebenikova, E. ; Prindle, Duncan J. ; Pruneau, Claude A. ; Radomski, S. ; Rai, Gulshan ; Ravel, Olivier ; Ray, Robert L. ; Razin, Sergej V. ; Reichhold, Dennis Michael ; Reid, Jeffrey G. ; Retiere, Fabrice ; Ridiger, Alexey ; Ritter, Hans Georg ; Roberts, Jabus B. ; Rogachevski, O. V. ; Roy, Christelle Sophie ; Russ, D. ; Rykov, Vladimir L. ; Sakrejda, Iwona ; Sandweiss, Jack H. ; Saulys, A. C. ; Savin, Igor A. ; Schambach, Joachim ; Scharenberg, Rolf Paul ; Schmitz, Norbert ; Schroeder, Lee S. ; Schüttauf, Andreas ; Seger, Janet Elizabeth ; Seliverstov, D. ; Seyboth, Peter ; Shestermanov, Konstantin E. ; Shimanskii, S. S. ; Shvetcov, V. S. ; Skoro, Goran P. ; Smirnov, Nikolai ; Snellings, Raimond ; Sowinski, James ; Spinka, Harold. M. ; Srivastava, Brijesh Kumar ; Stephenson, Edward J. ; Stock, Reinhard ; Stolpovsky, Alexander ; Strichanov, Michail Nikolaevič ; Stringfellow, Blair ; Ströbele, Herbert ; Struck, Christof ; Suaide, Alexandre Alarcon do Passo ; Sugarbaker, Evan R. ; Suire, Christophe Pierre ; Symons, Timothy James MacNeil ; Toledo, Alejandro Szanto de ; Szarwas, Piotr ; Takahashi, Jun ; Tang, Aihong ; Thomas, James H. ; Tikhomirov, V. ; Trainor, Thomas A. ; Trentalange, Stephen ; Tokarev, Mikhail V. ; Tonjes, Marguerite Belt ; Trofimov, V. ; Tsai, Oleg D. ; Turner, K. ; Ullrich, Thomas ; Underwood, David G. ; Van Buren, Gene ; VanderMolen, A. M. ; Vanyashin, A. ; Vasilevski, I. M. ; Vasiliev, Alexander Nikolaevich ; Vigdor, Steven E. ; Voloshin, Sergei ; Wang, F. ; Ward, H. ; Wells, R. ; Wenaus, T. ; Westfall, Gary D. ; Whitten, Charles A. ; Wieman, Howard ; Willson, Robert ; Wissink, Scott W. ; Witt, Richard ; Xu, N. ; Xu, Z. ; Yakutin, A. E. ; Yamamoto, E. ; Yang, J. ; Yepes, Pablo P. ; Yokosawa, A. ; Yurevich, Vladimir Yurevich ; Zanevski, Y. V. ; Zhang, J. ; Zhang, W. M. ; Zoulkarneev, R. ; Zubarev, A. N.
We report results on the ratio of midrapidity antiproton-to-proton yields in Au+Au collisions at sqrt[sNN] = 130 GeV per nucleon pair as measured by the STAR experiment at RHIC. Within the rapidity and transverse momentum range of | y|<0.5 and 0.4<pt<1.0 GeV/c, the ratio is essentially independent of either transverse momentum or rapidity, with an average of 0.65±0.01(stat)±0.07(syst) for minimum bias collisions. Within errors, no strong centrality dependence is observed. The results indicate that at this RHIC energy, although the p-p-bar pair production becomes important at midrapidity, a significant excess of baryons over antibaryons is still present.
Bowen, Tom ; Cicardi, Marco ; Farkas, Henriette ; Bork, Konrad ; Longhurst, Hilary ; Zuraw, Bruce ; Aygören-Pürsün, Emel ; Craig, Timothy ; Binkley, Karen ; Hebert, Jacques ; Ritchie, Bruce ; Bouillet, Laurence ; Betschel, Stephen ; Cogar, Della ; Dean, John ; Devaraj, Ramachand ; Hamed, Azza ; Kamra, Palinder ; Keith, Paul K. ; Lacuesta, Gina ; Leith, Eric ; Lyons, Harriet ; Mace, Sean ; Mako, Barbara ; Neurath, Doris ; Poon, Man-Chiu ; Rivard, Georges-Etienne ; Schellenberg, Robert ; Rowan, Dereth ; Rowe, Anne ; Stark, Donald ; Sur, Smeeksha ; Tsai, Ellie ; Warrington, Richard ; Waserman, Susan ; Ameratunga, Rohan ; Bernstein, Jonathan A. ; Björkander, Janne ; Brosz, Kristylea ; Brosz, John ; Bygum, Anette ; Caballero, Teresa ; Frank, Mike ; Fust, George ; Harmat, George ; Kanani, Amin ; Kreuz, Wolfhart ; Levi, Marcel M. ; Li, Henry ; Martinez Saguer, Inmaculada ; Moldovan, Dumitru ; Nagy, Istvan ; Nielsen, Erik W. ; Nordenfelt, Patrik ; Reshef, Avner ; Rusicke, Eva ; Smith-Foltz, Sarah ; Späth, Peter ; Varga, Lilian ; Xiang, Zhi Yu
Background We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) and updated this as Hereditary angioedema: a current state-of-the-art review: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Objective To update the International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema (circa 2010). Methods The Canadian Hereditary Angioedema Network (CHAEN)/Reseau Canadien d'angioedeme hereditaire (RCAH) (www.haecanada.com) and cosponsors University of Calgary and the Canadian Society of Allergy and Clinical Immunology (with an unrestricted educational grant from CSL Behring) held our third Conference May 15th to 16th, 2010 in Toronto Canada to update our consensus approach. The Consensus document was reviewed at the meeting and then circulated for review. Results This manuscript is the 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema that resulted from that conference. Conclusions Consensus approach is only an interim guide to a complex disorder such as HAE and should be replaced as soon as possible with large phase III and IV clinical trials, meta analyses, and using data base registry validation of approaches including quality of life and cost benefit analyses, followed by large head-to-head clinical trials and then evidence-based guidelines and standards for HAE disease management.
Betschel, Stephen ; Badiou, Jacquie ; Binkley, Karen ; Hébert, Jacques ; Kanani, Amin ; Keith, Paul K. ; Lacuesta, Gina ; Yang, Bill ; Aygören-Pürsün, Emel ; Bernstein, Jonathan A. ; Bork, Konrad ; Caballero, Teresa ; Cicardi, Marco ; Craig, Timothy ; Farkas, Henriette ; Longhurst, Hilary ; Zuraw, Bruce ; Boysen, Henrik B. ; Borici-Mazi, Rozita ; Bowen, Tom ; Dallas, Karen ; Dean, John ; Dean, John ; Lang-Robertson, Kelly ; Laramée, Benoît ; Leith, Eric ; Mace, Sean ; McCusker, Christine ; Moote, Bill ; Poon, Man-Chiu ; Ritchie, Bruce ; Stark, Donald ; Sussman, Gordon ; Waserman, Susan
Hereditary angioedema (HAE) is a disease which is associated with random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased Health Related Quality of Life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada is neither optimal nor uniform across the country. It lags behind other countries where there are more organized models for HAE management, and where additional therapeutic options are licensed and available for use. The objective of this guideline is to provide graded recommendations for the management of patients in Canada with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. It is anticipated that by providing this guideline to caregivers, policy makers, patients and their advocates, that there will be an improved understanding of the current recommendations regarding management of HAE and the factors that need to be considered when choosing therapies and treatment plans for individual patients. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency physicians, gastroenterologists, dentists and otolaryngologists, who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.
Craig, Timothy ; Zuraw, Bruce ; Longhurst, Hilary ; Cicardi, Marco ; Bork, Konrad ; Grattan, Clive ; Katelaris, Constance ; Sussman, Gordon ; Keith, Paul K. ; Yang, William ; Hébert, Jacques ; Hanzlikova, Jana ; Staubach-Renz, Petra ; Martinez Saguer, Inmaculada ; Magerl, Markus ; Aygören-Pürsün, Emel ; Farkas, Henriette ; Reshef, Avner ; Kivity, Shmuel ; Neri, Sergio ; Crișan, Ioana ; Caballero, Teresa ; Baeza, María Luisa ; Hernandez, Maria Dolores ; Li, Henry ; Lumry, William ; Bernstein, Jonathan A. ; Hussain, Iftikar ; Anderson, John ; Schwartz, Lawrence B. ; Jacobs, Joshua ; Manning, Michael ; Levy, Donald ; Riedl, Marc A. ; Christiansen, Sandra ; Feuersenger, Henrike ; Pragst, Ingo ; Mycroft, Sarah ; Pawaskar, Dipti ; Jacobs, Iris
Background: For the prevention of attacks of hereditary angioedema (HAE), the efficacy and safety of subcutaneous human C1-esterase inhibitor (C1-INH[SC]; HAEGARDA®, CSL Behring) was established in the 16-week COMPACT trial.
Objective: To assess the long-term safety, occurrence of angioedema attacks, and use of rescue medication with C1-INH(SC).
Methods: Open-label, randomised, parallel-arm extension of COMPACT across 11 countries. Patients with frequent angioedema attacks, either study treatment-naïve or who had completed the COMPACT trial, were randomly assigned (1:1) to 40 IU/kg or 60 IU/kg C1-INH(SC) twice per week, with conditional up-titration to optimise prophylaxis. ClinicalTrials.gov registration: NCT02316353.
Results: 126 patients with a monthly attack rate of 4.3 in 3 months prior to entry in the COMPACT program were enrolled and treated for a mean of 1·5 years; 44 patients (34·9%) had >2 years exposure. Median steady-state C1-INH functional activity increased to a maximum of 73.0% with 60 IU/kg. Incidence of adverse events was low and similar in both dose groups (11·3 and 8·5 events per patient-year for 40 IU/kg and 60 IU/kg, respectively). For 40 IU/kg and 60 IU/kg, median annualised attack rates were 1·3 and 1·0, respectively and median rescue medication use was 0.2 and 0.0 times per year, respectively. Of 23 patients receiving 60 IU/kg for >2 years, 19 (83%) were attack-free during months 25–30 of treatment.
Conclusion: In patients with frequent HAE attacks, long-term replacement therapy with C1-INH(SC) is safe and exhibits a substantial and sustained prophylactic effect, with the vast majority of patients becoming free from debilitating disease symptoms.