title: [Biological investigation of kinin-mediated angioedema].
abstract: Kinin-mediated angioedema results from accumulation of kinins, vasoactive and vasopermeant peptides, on the vascular endothelium. The disease is characterized by sudden episodes of swelling in the subcutaneous and submucosal tissues; the edema may occur spontaneously or it may be precipitated by triggering factors such as physical or emotional stress, or certain medicines. The characterization of kinin formation and catabolism systems helps improve knowledge of the aetiopathogenic mechanisms involved and provides the basis for classification of kinin-mediated angioedema conditions; thus, we may distinguish between angioedema with C1 inhibitor deficiency, whether inherited or acquired, and angioedema with normal C1 inhibitor activity, associated with increased kinin-forming activity or deficiency in kinin catabolism enzymes. In support of the clinical diagnosis, the physician may request laboratory investigation for a functional and molecular definition of the disease. Laboratory diagnosis is based on the characterization of: (1) kinin production control by C1 inhibitor investigation (function, antigen levels and circulating species); (2) kinin production (kininogenase activity, kininogen cleavage species); and (3) kinin catabolism enzymes (aminopeptidase P, carboxypeptidase N, angiotensin-I converting enzyme and dipeptidyl peptidase IV). An abnormal biological phenotype is supported by examination of susceptibility genes (SERPING1, F12 and XPNPEP2) and mutation segregation in the families.
authors: Exploration fonctionnelle de l'angiœdème, CHU Grenoble, CS 10217, 38043 Grenoble, France; Université Joseph Fourier, GREPI/AGIM CNRS FRE 3405, 38043 Grenoble cedex 9, France. Electronic address: FDefendi@chu-grenoble.fr.
authors: F
authors: F
authors: Defendi
authors: Exploration fonctionnelle de l'angiœdème, CHU Grenoble, CS 10217, 38043 Grenoble, France; Université Joseph Fourier, GREPI/AGIM CNRS FRE 3405, 38043 Grenoble cedex 9, France.
authors: D
authors: D
authors: Charignon
authors: Exploration fonctionnelle de l'angiœdème, CHU Grenoble, CS 10217, 38043 Grenoble, France; Université Joseph Fourier, GREPI/AGIM CNRS FRE 3405, 38043 Grenoble cedex 9, France.
authors: A
authors: A
authors: Ghannam
authors: Laboratoire d'immunologie, CHU Grenoble, CS 10217, 38043 Grenoble, France.
authors: D
authors: D
authors: Ponard
authors: Exploration fonctionnelle de l'angiœdème, CHU Grenoble, CS 10217, 38043 Grenoble, France; Université Joseph Fourier, GREPI/AGIM CNRS FRE 3405, 38043 Grenoble cedex 9, France.
authors: C
authors: C
authors: Drouet
mesh_terms: D000799:Angioedema / Q000097:blood* / Q000145:classification / Q000175:diagnosis* / Q000209:etiology; D003174:Complement C1 Inactivator Proteins / Q000032:analysis; D050718:Complement C1 Inhibitor Protein; D003663:Decision Trees; D006801:Humans; D007705:Kinins / Q000097:blood* / Q000502:physiology
chemical_list: D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D007705:Kinins; C469952:SERPING1 protein, human
keywords: Angioedema; Angiœdème; Bradykinin; Bradykinine; C1 inhibiteur; C1 inhibitor; Contact Phase; Kinin formation; Kininases; Kininoformation; Phase contact