title: [Angioedema is caused by a defect in C1-inhibitor synthesis].
abstract: Deficit of the first component of complement inhibitor (C1-inhibitor, C1-inh) may clinically be manifested as angioedema. The disease is characterized by episodic swellings of mucosa and subcutaneous tissue at different locations of the body. Laryngeal swelling can be life-threatening. The major mediators of edema are discussed to be bradykinin and C2b derived peptides. These mediators increase capillary permeability. Antifibrinolytic agents (aminocaproic acid, tranexamic acid) and attenuated androgens (danazol or stanazolol) are used for prophylaxis. Prolonged use both of them might result in more or less severe side effects. In experiments in vitro it has been shown that IFN-gamma, IL-1, IL-6 have a stimulatory effect on C1-inh synthesis. We want to verify the practical use of probiotics as natural inductors of IFN-gamma synthesis for elevating C1-inh level.
authors.affiliation: Zakład Immunologii Klinicznej Instytutu-Pomnika Centrum Zdrowia Dziecka w Warszawie. kmadal@czd.waw.pl
authors.forename: H
authors.initials: H
authors.lastname: Jaworska
authors.forename: H
authors.initials: H
authors.lastname: Gregorek
authors.forename: K
authors.initials: K
authors.lastname: Madaliński
mesh_terms: D000293:Adolescent; D000799:Angioedema / Q000188:drug therapy / Q000209:etiology* / Q000378:metabolism*; D000818:Animals; D000933:Antifibrinolytic Agents / Q000494:pharmacology; D001920:Bradykinin / Q000378:metabolism; D002199:Capillary Permeability / Q000187:drug effects / Q000502:physiology; D002478:Cells, Cultured; D002648:Child; D002675:Child, Preschool; D003174:Complement C1 Inactivator Proteins / Q000187:drug effects / Q000378:metabolism*; D003175:Complement C2 / Q000378:metabolism; D050679:Complement C2b; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D019936:Probiotics / Q000627:therapeutic use
chemical_list: D000933:Antifibrinolytic Agents; D003174:Complement C1 Inactivator Proteins; D003175:Complement C2; D050679:Complement C2b; D001920:Bradykinin
vernacular_title: Obrzek naczynioruchowy zalezny od defektu syntezy C1-inhibitora.