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Hereditary Angioedema with Normal C1 Inhibitor: an Updated International Consensus Paper on Diagnosis, Pathophysiology, and Treatment.

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Title: Hereditary Angioedema with Normal C1 Inhibitor: an Updated International Consensus Paper on Diagnosis, Pathophysiology, and Treatment.
Authors: Zuraw BL; Department of Medicine, Division of Allergy & Immunology, University of California San Diego, 9500 Gilman Drive, Mail Code 0732, La Jolla, CA, 92093, USA. bzuraw@health.ucsd.edu.; Medicine Service, San Diego VA Healthcare, San Diego, USA. bzuraw@health.ucsd.edu., Bork K; Department of Dermatology, University Medical Center, Johannes Gutenberg University Mainz, Mainz, Germany., Bouillet L; University Grenoble Alpes, T-RAIG Unit, CNRS, UMR 5525, TIMC, Grenoble, France.; French National Reference Center for Angioedema (CREAK), Internal Medicine Department, Grenoble University Hospital, Grenoble, France., Christiansen SC; Department of Medicine, Division of Allergy & Immunology, University of California San Diego, 9500 Gilman Drive, Mail Code 0732, La Jolla, CA, 92093, USA., Farkas H; Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary., Germenis AE; Department of Immunology & Histocompatibility, School of Medicine, University of Thessaly, Larissa, Greece., Grumach AS; Angioedema Center of Reference and Excellence (ACARE), Centro Universitario Faculdade de Medicina ABC (CEUFMABC), São Paulo, Brazil., Kaplan A; Medical University of South Carolina, Charleston, SC, USA., López-Lera A; Hospital La Paz Institute for Health Research (IdiPAZ), CIBERER (U754), Madrid, Spain., Magerl M; Angioedema Center of Reference and Excellence (ACARE), Institute of Allergology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, Berlin, Germany.; Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany., Riedl MA; Department of Medicine, Division of Allergy & Immunology, University of California San Diego, 9500 Gilman Drive, Mail Code 0732, La Jolla, CA, 92093, USA., Adatia A; Division of Pulmonary Medicine, Department of Medicine, University of Alberta, Edmonton, AB, Canada., Banerji A; Department of Medicine, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, MA, USA., Betschel S; Division of Clinical Immunology and Allergy, University of Toronto, Toronto, ON, Canada., Boccon-Gibod I; University Grenoble Alpes, T-RAIG Unit, CNRS, UMR 5525, TIMC, Grenoble, France., Bova M; Division of Internal Medicine 2, Department of Medicine and Medical Specialties, A. Cardarelli Hospital, Naples, Italy., Boysen HB; HAE International (HAEi), Fairfax, VA, USA.; US Hereditary Angioedema Association (HAEA), Fairfax, VA, USA., Caballero T; Hospital La Paz Institute for Health Research (IdiPAZ), CIBERER (U754), Madrid, Spain.; Department of Allergy, La Paz University Hospital, Madrid, Spain., Cancian M; Department of Systems Medicine, University of Padua, Padua, Italy., Castaldo AJ; HAE International (HAEi), Fairfax, VA, USA.; US Hereditary Angioedema Association (HAEA), Fairfax, VA, USA., Cohn DM; Department of Vascular Medicine, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands., Corcoran D; HAE International (HAEi), Fairfax, VA, USA.; US Hereditary Angioedema Association (HAEA), Fairfax, VA, USA., Drouet C; Institut Cochin, Université Paris Cité, INSERM U1016, Paris, France., Fukunaga A; Department of Dermatology, Division of Medicine for Function and Morphology of Sensory Organs, Faculty of Medicine, Osaka Medical and Pharmaceutical University, Takatsuki-City, Osaka, Japan., Hide M; Department of Dermatology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan.; Department of Dermatology, Hiroshima University, Hiroshima, Japan., Katelaris CH; Immunology & Allergy Unit, Dept of Medicine, Campbelltown Hospital and Western Sydney University, Sydney, Australia., Li PH; Division of Rheumatology and Clinical Immunology, Department of Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, Hong Kong., Longhurst H; Department of Medicine, University of Auckland and Department of Immunology, Auckland City Hospital, Auckland, New Zealand., Peter J; Division of Allergy and Clinical Immunology, Department of Medicine, Groote Schuur Hospital, University of Cape Town, Cape Town, South Africa.; Allergy and Immunology Unit, University of Cape Town Lung Institute, Cape Town, South Africa., Psarros F; Department of Allergy, Athens Naval Hospital, Athens, Greece., Reshef A; Angioedema Research Unit, Barzilai University Medical Center, Ashkelon, Israel., Ritchie B; Division of Hematology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada., Selva CN; US Hereditary Angioedema Association (HAEA), Fairfax, VA, USA., Zanichelli A; Operative Unit of Medicine, Angioedema Center, IRCCS Policlinico San Donato, San Donato Milanese, Milan, Italy.; Dipartimento Di Scienze Biomediche Per La Salute, University of Milan, Milan, Italy., Maurer M; Angioedema Center of Reference and Excellence (ACARE), Institute of Allergology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, Berlin, Germany.; Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany.
Source: Clinical reviews in allergy & immunology [Clin Rev Allergy Immunol] 2025 Mar 07; Vol. 68 (1), pp. 24. Date of Electronic Publication: 2025 Mar 07.
Publication Type: Journal Article; Review; Consensus Statement
Journal Info: Publisher: Humana Press Country of Publication: United States NLM ID: 9504368 Publication Model: Electronic Cited Medium: Internet ISSN: 1559-0267 (Electronic) Linking ISSN: 10800549 NLM ISO Abbreviation: Clin Rev Allergy Immunol Subsets: MEDLINE
Database: MEDLINE Ultimate
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  Data: Hereditary Angioedema with Normal C1 Inhibitor: an Updated International Consensus Paper on Diagnosis, Pathophysiology, and Treatment.
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  Data: <searchLink fieldCode="AU" term="%22Zuraw+BL%22">Zuraw BL</searchLink>; Department of Medicine, Division of Allergy & Immunology, University of California San Diego, 9500 Gilman Drive, Mail Code 0732, La Jolla, CA, 92093, USA. bzuraw@health.ucsd.edu.; Medicine Service, San Diego VA Healthcare, San Diego, USA. bzuraw@health.ucsd.edu.<br /><searchLink fieldCode="AU" term="%22Bork+K%22">Bork K</searchLink>; Department of Dermatology, University Medical Center, Johannes Gutenberg University Mainz, Mainz, Germany.<br /><searchLink fieldCode="AU" term="%22Bouillet+L%22">Bouillet L</searchLink>; University Grenoble Alpes, T-RAIG Unit, CNRS, UMR 5525, TIMC, Grenoble, France.; French National Reference Center for Angioedema (CREAK), Internal Medicine Department, Grenoble University Hospital, Grenoble, France.<br /><searchLink fieldCode="AU" term="%22Christiansen+SC%22">Christiansen SC</searchLink>; Department of Medicine, Division of Allergy & Immunology, University of California San Diego, 9500 Gilman Drive, Mail Code 0732, La Jolla, CA, 92093, USA.<br /><searchLink fieldCode="AU" term="%22Farkas+H%22">Farkas H</searchLink>; Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.<br /><searchLink fieldCode="AU" term="%22Germenis+AE%22">Germenis AE</searchLink>; Department of Immunology & Histocompatibility, School of Medicine, University of Thessaly, Larissa, Greece.<br /><searchLink fieldCode="AU" term="%22Grumach+AS%22">Grumach AS</searchLink>; Angioedema Center of Reference and Excellence (ACARE), Centro Universitario Faculdade de Medicina ABC (CEUFMABC), São Paulo, Brazil.<br /><searchLink fieldCode="AU" term="%22Kaplan+A%22">Kaplan A</searchLink>; Medical University of South Carolina, Charleston, SC, USA.<br /><searchLink fieldCode="AU" term="%22López-Lera+A%22">López-Lera A</searchLink>; Hospital La Paz Institute for Health Research (IdiPAZ), CIBERER (U754), Madrid, Spain.<br /><searchLink fieldCode="AU" term="%22Magerl+M%22">Magerl M</searchLink>; Angioedema Center of Reference and Excellence (ACARE), Institute of Allergology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, Berlin, Germany.; Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany.<br /><searchLink fieldCode="AU" term="%22Riedl+MA%22">Riedl MA</searchLink>; Department of Medicine, Division of Allergy & Immunology, University of California San Diego, 9500 Gilman Drive, Mail Code 0732, La Jolla, CA, 92093, USA.<br /><searchLink fieldCode="AU" term="%22Adatia+A%22">Adatia A</searchLink>; Division of Pulmonary Medicine, Department of Medicine, University of Alberta, Edmonton, AB, Canada.<br /><searchLink fieldCode="AU" term="%22Banerji+A%22">Banerji A</searchLink>; Department of Medicine, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, MA, USA.<br /><searchLink fieldCode="AU" term="%22Betschel+S%22">Betschel S</searchLink>; Division of Clinical Immunology and Allergy, University of Toronto, Toronto, ON, Canada.<br /><searchLink fieldCode="AU" term="%22Boccon-Gibod+I%22">Boccon-Gibod I</searchLink>; University Grenoble Alpes, T-RAIG Unit, CNRS, UMR 5525, TIMC, Grenoble, France.<br /><searchLink fieldCode="AU" term="%22Bova+M%22">Bova M</searchLink>; Division of Internal Medicine 2, Department of Medicine and Medical Specialties, A. Cardarelli Hospital, Naples, Italy.<br /><searchLink fieldCode="AU" term="%22Boysen+HB%22">Boysen HB</searchLink>; HAE International (HAEi), Fairfax, VA, USA.; US Hereditary Angioedema Association (HAEA), Fairfax, VA, USA.<br /><searchLink fieldCode="AU" term="%22Caballero+T%22">Caballero T</searchLink>; Hospital La Paz Institute for Health Research (IdiPAZ), CIBERER (U754), Madrid, Spain.; Department of Allergy, La Paz University Hospital, Madrid, Spain.<br /><searchLink fieldCode="AU" term="%22Cancian+M%22">Cancian M</searchLink>; Department of Systems Medicine, University of Padua, Padua, Italy.<br /><searchLink fieldCode="AU" term="%22Castaldo+AJ%22">Castaldo AJ</searchLink>; HAE International (HAEi), Fairfax, VA, USA.; US Hereditary Angioedema Association (HAEA), Fairfax, VA, USA.<br /><searchLink fieldCode="AU" term="%22Cohn+DM%22">Cohn DM</searchLink>; Department of Vascular Medicine, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands.<br /><searchLink fieldCode="AU" term="%22Corcoran+D%22">Corcoran D</searchLink>; HAE International (HAEi), Fairfax, VA, USA.; US Hereditary Angioedema Association (HAEA), Fairfax, VA, USA.<br /><searchLink fieldCode="AU" term="%22Drouet+C%22">Drouet C</searchLink>; Institut Cochin, Université Paris Cité, INSERM U1016, Paris, France.<br /><searchLink fieldCode="AU" term="%22Fukunaga+A%22">Fukunaga A</searchLink>; Department of Dermatology, Division of Medicine for Function and Morphology of Sensory Organs, Faculty of Medicine, Osaka Medical and Pharmaceutical University, Takatsuki-City, Osaka, Japan.<br /><searchLink fieldCode="AU" term="%22Hide+M%22">Hide M</searchLink>; Department of Dermatology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan.; Department of Dermatology, Hiroshima University, Hiroshima, Japan.<br /><searchLink fieldCode="AU" term="%22Katelaris+CH%22">Katelaris CH</searchLink>; Immunology & Allergy Unit, Dept of Medicine, Campbelltown Hospital and Western Sydney University, Sydney, Australia.<br /><searchLink fieldCode="AU" term="%22Li+PH%22">Li PH</searchLink>; Division of Rheumatology and Clinical Immunology, Department of Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, Hong Kong.<br /><searchLink fieldCode="AU" term="%22Longhurst+H%22">Longhurst H</searchLink>; Department of Medicine, University of Auckland and Department of Immunology, Auckland City Hospital, Auckland, New Zealand.<br /><searchLink fieldCode="AU" term="%22Peter+J%22">Peter J</searchLink>; Division of Allergy and Clinical Immunology, Department of Medicine, Groote Schuur Hospital, University of Cape Town, Cape Town, South Africa.; Allergy and Immunology Unit, University of Cape Town Lung Institute, Cape Town, South Africa.<br /><searchLink fieldCode="AU" term="%22Psarros+F%22">Psarros F</searchLink>; Department of Allergy, Athens Naval Hospital, Athens, Greece.<br /><searchLink fieldCode="AU" term="%22Reshef+A%22">Reshef A</searchLink>; Angioedema Research Unit, Barzilai University Medical Center, Ashkelon, Israel.<br /><searchLink fieldCode="AU" term="%22Ritchie+B%22">Ritchie B</searchLink>; Division of Hematology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.<br /><searchLink fieldCode="AU" term="%22Selva+CN%22">Selva CN</searchLink>; US Hereditary Angioedema Association (HAEA), Fairfax, VA, USA.<br /><searchLink fieldCode="AU" term="%22Zanichelli+A%22">Zanichelli A</searchLink>; Operative Unit of Medicine, Angioedema Center, IRCCS Policlinico San Donato, San Donato Milanese, Milan, Italy.; Dipartimento Di Scienze Biomediche Per La Salute, University of Milan, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Maurer+M%22">Maurer M</searchLink>; Angioedema Center of Reference and Excellence (ACARE), Institute of Allergology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, Berlin, Germany.; Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany.
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  Data: <searchLink fieldCode="JN" term="%229504368%22">Clinical reviews in allergy & immunology</searchLink> [Clin Rev Allergy Immunol] 2025 Mar 07; Vol. 68 (1), pp. 24. <i>Date of Electronic Publication: </i>2025 Mar 07.
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