![]() ![]() Most notably, it includes cumulative genetic investigations multinational laboratory diagnosis recommendations current pathogenesis hypotheses suggested prophylaxis and acute attack treatment, including home treatment future treatment options and analysis of patient subpopulations, including pediatric patients and patients whose angioedema worsened during pregnancy or hormone administration. This supplement contains work presented at the third workshop and expanded content toward a definitive picture of angioedema in the absence of allergy. The biennial C1 Esterase Inhibitor Deficiency Workshops resulted from a European initiative for better knowledge and treatment of HAE and related diseases. Low awareness and resemblance to other disorders often delay diagnosis despite availability of C1-INH replacement in some countries, no approved, safe acute attack therapy exists in the United States. Resulting from mutations affecting C1 esterase inhibitor (C1-INH), inhibitor of the first complement system component, attacks are not histamine-mediated and do not respond to antihistamines or corticosteroids. Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema. Nuijens, MD, PhD, Caroline O'Grady, RGN, Emanuela Pappalardo, PhD, ∗ Vincenzo Penna, Carlo Perricone, Roberto Perricone, MD, Ursula Rauch, Olga Roche, ∗ Eva Rusicke, Peter J Späth, PhD, FAMH, George Szendei, MD, Edit Takács, MD, Attila Tordai, MD, PhD, ∗ Lennart Truedsson, MD, PhD, ∗ Lilian Varga, PhD, ∗ Beáta Visy, MD, Kayla Williams, BS, MA, MFA, Andrea Zanichelli, and Lorenza Zingale, MD ∗ ![]() Longhurst, MA, MRCP, PhD, MRCPath, ∗ Margarita Lopez-Trascasa, MD, PhD, ∗ Inmaculada Martinez-Saguer, MD, Nicole Monnier, PhD, István Nagy, Éva Németh, Erik Waage Nielsen, MD, PhD, ∗ Jan H. Kaposi, MD, PhD, István Karádi, MD, PhD, DSc, Arianna Kitzinger, Tímea Kollár, MD, Wolfhart Kreuz, MD, PhD, Peter Lakatos, MD, PhD, DSc, Hilary J. ![]() Erik Hack, MD, PhD, ∗ George Harmat, MD, PhD, John Jakenfelds, MD, Mathias Juers, MD, Lajos Kalmár, Pál N. Binkley, MD, FRCPC, Alvaro Blanch, ∗ Konrad Bork, MD, ∗ Laurence Bouillet, MD, CCA, ∗ Christoph Bucher, MD, ∗ Anthony J Castaldo, MPA, Marco Cicardi, MD, ∗ Alvin E Davis, III, MD, Caterina De Carolis, MD, Christian Drouet, PhD, ∗ Christiane Duponchel, Henriette Farkas, MD, PhD, ∗ Kálmán Fáy, MD, Béla Fekete, MD, PhD, DSc, Bettina Fischer, MD, Luigi Fontana, MD, George Füst, MD, PhD, DSc, ∗ Roberto Giacomelli, MD, Albrecht Gröner, PhD, C. Angelo Agostoni, MD, Emel Aygören-Pürsün, MD, Karen E. ![]()
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