Hereditary angioedema with normal C1 inhibitor: clinical characteristics and treatment response with plasma-derived human C1 inhibitor concentrate (Berinert<sup>®</sup>) in a French cohort.

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第一作者： Laurence,Bouillet

第一单位： Univ. Grenoble Alpes, Joint unit 1036 INSERM-CEA-CNRS, CREAK, Department of Internal Medicine, PPMGC, CHU Grenoble, Grenoble, France, National Reference Centre for Angioedema, CREAK, France.

作者： Laurence,Bouillet ^[1] ; Isabelle,Boccon-Gibod ^[1] ; Anne,Gompel ^[2] ; Bernard,Floccard ^[3] ; Ludovic,Martin ^[4] ; Claire,Blanchard-Delaunay ^[5] ; David,Launay ^[6] ; Olivier,Fain ^[7]

作者单位： Univ. Grenoble Alpes, Joint unit 1036 INSERM-CEA-CNRS, CREAK, Department of Internal Medicine, PPMGC, CHU Grenoble, Grenoble, France, National Reference Centre for Angioedema, CREAK, France. ^[1] National Reference Centre for Angioedema, CREAK, France, Endocrine Gynaecology, Paris Descartes University, University Hospital Centre, Paris, France. ^[2] National Reference Centre for Angioedema, CREAK, France, Intensive Care Unit, Edouard Herriot Hospital, Hospices Civils de Lyon, Lyon, France. ^[3] National Reference Centre for Angioedema, CREAK, France, Department of Dermatology, CHU Angers, Angers, France. ^[4] National Reference Centre for Angioedema, CREAK, France, Department of Internal Medicine, CH Niort, France. ^[5] National Reference Centre for Angioedema, CREAK, France, Université Lille Nord de France, Department of Internal Medicine, National Reference Centre for Autoimmune and Systemic Rare Diseases, Hospital Claude-Huriez, CHRU Lille, France. ^[6] National Reference Centre for Angioedema, CREAK, France, Department of Internal Medicine, Saint Antoine University Hospital, AP-HP, Paris, France. ^[7]

关键词 Berinert?C1 inhibitor drug safety hereditary angioedema off label pharmaco-epidemiological registry