[Hereditary angioneurotic edema. Recent progress in diagnosis and treatment. Importance of a national registry]

Abstract

The hereditary angioneurotic oedema is an exceptional disease of deadly risk from glottic oedema. The diagnosis is based on a very low serum C4, with collapsed C1 INH for a patient who has repeated attacks of Quincke's oedema or pseudo occlusive digestive crisis. These crisis are prevented by taking anabolic steroids and must be treated when they are serious by intravenous C1 INH. The recent contributions of the immunogenetic and the interest of a national registry are underlined.