遗传性血管神经性喉水肿1例

患者，女，60岁，因声音嘶哑1d于2013年2月8日来院就诊。患者约30年前起反复声音嘶哑、咽部异物感，偶伴呼吸困难及身体其他部位水肿，每年发作6～7次，每次持续3～5d。先后多家医院就诊，按喉部过敏性炎症给以激素治疗好转。

One case of hereditary angioneurotic laryngeal edema

Hereditary angioneurotic laryngeal edema（HALE） is an autosomal dominant hereditary disease in which there is a decrease or defect in the C1 inhibitor （Cl-INH）. The pathophysiology of HALE is characterized by recurrent spontaneous episodes of transient edema of the laryngeal mucose and submucosal tissue with remission at irregular. Patients may die because of a life-threatening acute upper airway obstruction caused by laryngeal edema. HALE was diagnosed on the clinical symptoms, family history,and markedly decreased serum C1-INH activity and C1-INH protein.