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 useABSTRACT
BACKGROUND/AIMS: In liver surgery, total clamping of the portal triad (Pringle's procedure) is commonly used, and sometimes causes liver failure. This study evaluated the effects of a bradykinin B2 receptor antagonist, FR173657 (FR), on ischemia-reperfusion injury during liver resection in dogs. METHODOLOGY: Experimental animals were divided into two groups. In the FR group (n=6), FR (100 nmol/kg/hr) was administered continuously via the portal vein from 30 min before the onset of ischemia until 2 hr after reperfusion. In the control group (n=6), vehicle was injected in the same manner. The right portal pedicle was clamped for 60 min, while the left portal branch was left patent to avoid portal congestion. Following reperfusion, the non-ischemic lobes were resected, and remnant liver function was evaluated. RESULTS: AST and ALT were significantly (p<0.05) lower in the FR group than in the control group. Hepatic tissue blood flow 30 min after reperfusion was significantly (p<0.05) higher in the FR group than in the control group. Histological tissue damage was mild, and polymorphonuclear neutrophil infiltration was significantly (p<0.05) reduced in the FR group compared with the control group. CONCLUSIONS: A bradykinin B2 receptor antagonist ameliorated the ischemia-reperfusion injury caused by Pringle's procedure during extended liver resection.
