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Ugeskr Laeger ; 174(34): 1894-8, 2012 Aug 20.
Article in Danish | MEDLINE | ID: mdl-22909569

ABSTRACT

Hereditary angio-oedema (HAE) is a rare genetic disease caused by deficiency of complement C1 inhibitor. It is characterised by recurrent episodes of subcutaneous or submucosal oedema typically involving the extremities, bowel, face or larynx. Within the latest years it has become evident that the active mediator of HAE attacks is an increased level of bradykinin and various new treatment modalities have been developed. The aim of this paper is to give an update from the Danish HAE Comprehensive Care Centre on current treatment possibilities and address some of the challenges when diagnosing HAE.


Subject(s)
Angioedemas, Hereditary/drug therapy , Bradykinin B2 Receptor Antagonists , Complement C1 Inactivator Proteins/therapeutic use , Adult , Angioedemas, Hereditary/complications , Angioedemas, Hereditary/diagnosis , Angioedemas, Hereditary/pathology , Bradykinin/administration & dosage , Bradykinin/analogs & derivatives , Bradykinin/therapeutic use , Complement C1 Inactivator Proteins/administration & dosage , Complement C1 Inactivator Proteins/genetics , Complement C1 Inactivator Proteins/metabolism , Complement C1 Inhibitor Protein/administration & dosage , Complement C1 Inhibitor Protein/therapeutic use , Complement Inactivating Agents/administration & dosage , Complement Inactivating Agents/therapeutic use , Diagnosis, Differential , Erythema/pathology , Female , Humans , Kallikreins/antagonists & inhibitors , Laryngeal Edema/etiology , Peptides/administration & dosage , Peptides/therapeutic use , Receptor, Bradykinin B2/administration & dosage , Receptor, Bradykinin B2/therapeutic use , Recombinant Proteins/administration & dosage , Recombinant Proteins/therapeutic use
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