title: [Hereditary angioneurotic edema (author's transl)].
abstract: Hereditary angioneurotic edema is a rare autosomally-inherited disease, which is caused by a deficiency or functional inactivation of C-1 inactivator in the complement system. The poor prognosis of the disease mandates that an early diagnosis be made and drug therapy begun to prevent a lethal outcome. The pathogenesis, clinical course and therapies available are detailed.
mesh_terms: D000799:Angioedema / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000276:immunology / Q000627:therapeutic use; D003613:Danazol / Q000627:therapeutic use; D006801:Humans; D008777:Methyltestosterone / Q000627:therapeutic use; D011379:Prognosis
chemical_list: D003174:Complement C1 Inactivator Proteins; D003613:Danazol; D008777:Methyltestosterone
vernacular_title: Das hereditäre angioneurotische Odem.