遺伝性血管神経性浮腫（hereditary angioneurotic edema, HANE）の1症例

Hereditary Angioneurotic Edema (HANE):Report of a Case

　はじめに

　遺伝性血管神経性浮腫（hereditary angioneuro-tic edema：以下，HANEと略）とは，補体第1成分阻止因子（以下，C1—INHと略）の減少により，種々の誘因で身体各部分に浮腫を生じる常染色体優性遺伝を示す疾患である。本邦では稀であり，現在までの報告例は28家系である。今回われわれは，口唇周囲と咽喉頭に浮腫を生じたHANEの症例を経験したので，若干の文献的考察を加えて報告する。

A 50-year-old male was complaining of a swell-ing of the lip and sore throat. He and his mother had a past history of frequent edema attack. Laboratoryexaminations revealed hypocomplementemia, marked depletion of the fourth component of com-plement and low level of C1-INH. Antibiotics and hydrocortisone sodium succinate were administered, and the swelling of the lip and larynx disappeared after 72 hours. In Japan, about 30 families of HANE have been reported. Most of these cases treated with hydrocortisone, danazol and tranexamic acid.