About: Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond

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About: Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond Goto Sponge NotDistinct Permalink

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type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
has title	Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond
Creator	Truedsson, Lennart Farkas, Henriette Varga, Lilian Perricone, Carlo Agostoni, Angelo Blanch, Alvaro Bork, Konrad Bouillet, Laurence Bucher, Christoph Cicardi, Marco De Carolis, Caterina Drouet, Christian Duponchel, Christiane Fischer, Bettina Fontana, Luigi Giacomelli, Roberto Harmat, George Jakenfelds, John Juers, Mathias Kitzinger, Arianna Kreuz, Wolfhart Lakatos, Peter Lopez-Trascasa, Margarita Martinez-Saguer, Inmaculada Monnier, Nicole Pappalardo, Emanuela Penna, Vincenzo Perricone, Roberto Rauch, Ursula Roche, Olga Rusicke, Eva Szendei, George Tordai, Attila Williams, Kayla Zanichelli, Andrea Zingale, Lorenza London, Ma, Switzerland, Lund, Washington, Aquila, L Aygö Ren-Pü Rsü, Emel Bern, Binkley, Karen Borromeo, Peschiera Cambridge, Székesféhérvar Canada, Rouen Castaldo, Anthony Davis, Alvin Di, Kará Fekete, La Fü St, George Germany, Giles, Grö, Albrecht Hack, C Kalmá, Lajos Kaposi, N Kollá, Tímea Leiden, The Lmá N Fá, Ká Longhurst, Hilary Main, Frankfut Netherlands, Chalfont Nielsen, Erik Nuijens, Jan O'grady, Caroline Rome, Milan Siersdorf, Aldenhoven Spä, Peter Ta Visy, Beá Toronto,
Source	Elsevier; Medline; PMC
abstract	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. 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. 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. The biennial C1 Esterase Inhibitor Deficiency Workshops resulted from a European initiative for better knowledge and treatment of HAE and related diseases. This supplement contains work presented at the third workshop and expanded content toward a definitive picture of angioedema in the absence of allergy. 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. Causes and management of acquired angioedema and a new type of angioedema with normal C1-INH are also discussed. Collaborative patient and physician efforts, crucial in rare diseases, are emphasized. This supplement seeks to raise awareness and aid diagnosis of HAE, optimize treatment for all patients, and provide a platform for further research in this rare, partially understood disorder.
has issue date	2004-09-30(xsd:dateTime)
bibo:doi	10.1016/j.jaci.2004.06.047
bibo:pmid	15356535
has license	els-covid
sha1sum (hex)	ee786c268711dc91475fd86bccb7662bb8a0994a
schema:url	https://doi.org/10.1016/j.jaci.2004.06.047
resource representing a document's title	Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond
has PubMed Central identifier	PMC7119155
has PubMed identifier	15356535
schema:publication	Journal of Allergy and Clinical Immunology
resource representing a document's body	covid:ee786c268711dc91475fd86bccb7662bb8a0994a#body_text
is schema:about of	named entity 'patient' named entity 'attack' named entity 'rare' named entity 'patients' named entity 'hypotheses' named entity 'This' named entity 'C1-INH' named entity 'administration' named entity 'secondary'

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