遺伝性血管神経性浮腫の1症例

A Case of Hereditary Angioneurotic Edema

　はじめに

　耳鼻咽喉科医が日常の臨床で遭遇する頭頸部浮腫疾患の多くはアレルギー性のクインケ浮腫であり，通常ステロイド，抗ヒスタミン剤などで治療可能である。今回われわれはステロイド，抗ヒスタミン剤に全く効果を示さず，3回の気管切開を必要とした喉頭浮腫症例を経験した。血清学的に補体第1成分阻止因子（C1—inactivator，C1—inhibitor：以下，C1—INHと略）の活性低下を示し，家族性は明らかでないものの遺伝性血管神経性浮腫（hereditary angioneurotic edema：以下，HANEと略）と診断されたため，若干の文献的考察を加えて報告する。

A 58-year-old woman presented with edema of the lower lip. She had recurrent attacks of acute edema of the face, arms and various parts of the trunk. Tracheotomy had been performed threetimes becase of life-threating laryngeal edema. Complement studies revealed low level of CH50, C4 and C1 inhibitor (-INH) activity with a normal C3 level. Although no hereditary trait could be traced in our patient, she was diagnosed as having heredi-tary angioneurotic edema based on recurrent edema attacks and complement disorders. Administration of C1-INH concentrate was very effective and la-ryngeal edema was well controlled without tra-cheotomy.