Hereditary angioedema: a bradykinin-mediated swelling disorder

TY - JOUR

T1 - Hereditary angioedema: a bradykinin-mediated swelling disorder

AU - Björkqvist, Jenny

AU - Sala-Cunill, Anna

AU - Renné, Thomas

PY - 2013/3/1

Y1 - 2013/3/1

N2 - Edema is tissue swelling and is a common symptom in a variety of diseases. Edema form due to accumulation of fluids, either through reduced drainage or increased vascular permeability. There are multiple vascular signalling pathways that regulate vessel permeability. An important mediator that increases vascular leak is the peptide hormone bradykinin, which is the principal agent in the swelling disorder hereditary angioedema. The disease is autosomal dominant inherited and presents clinically with recurrent episodes of acute swelling that can be life-threatening involving the skin, the oropharyngeal, laryngeal, and gastrointestinal mucosa. Three different types of hereditary angiodema exist in patients. The review summarises current knowledge on the pathophysiology of hereditary angiodema and focuses on recent experimental and pharmacological findings that have led to a better understanding and new treatments for the disease.

AB - Edema is tissue swelling and is a common symptom in a variety of diseases. Edema form due to accumulation of fluids, either through reduced drainage or increased vascular permeability. There are multiple vascular signalling pathways that regulate vessel permeability. An important mediator that increases vascular leak is the peptide hormone bradykinin, which is the principal agent in the swelling disorder hereditary angioedema. The disease is autosomal dominant inherited and presents clinically with recurrent episodes of acute swelling that can be life-threatening involving the skin, the oropharyngeal, laryngeal, and gastrointestinal mucosa. Three different types of hereditary angiodema exist in patients. The review summarises current knowledge on the pathophysiology of hereditary angiodema and focuses on recent experimental and pharmacological findings that have led to a better understanding and new treatments for the disease.

KW - Angioedemas, Hereditary

KW - Animals

KW - Anticoagulants

KW - Bradykinin

KW - Capillary Permeability

KW - Edema

KW - Heparin

KW - Humans

KW - Inflammation

KW - Kinins

KW - Polyphosphates

KW - Protein Folding

KW - Signal Transduction

U2 - 10.1160/TH12-08-0549

DO - 10.1160/TH12-08-0549

M3 - SCORING: Journal article

C2 - 23306453

VL - 109

SP - 368

EP - 374

JO - THROMB HAEMOSTASIS

JF - THROMB HAEMOSTASIS

SN - 0340-6245

IS - 3

ER -