The Challenge of Hereditary Angioneurotic Edema

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Abstract
Sudden death from airway obstruction is a constant and dreadful threat to patients with hereditary angioneurotic edema. Patients with this disorder are subject to repeated episodes of localized noninflammatory subepithelial edema, which may be provoked by physical or emotional trauma. It is inherited as an autosomal dominant trait, characterized by a deficiency in the functions of the inhibitor of the activated first component of complement, C1̄. The extraordinary clinical picture in these patients was beautifully described by Sir William Osler, but as early as 1851 Nathaniel Hawthorne doubtless wrote of this disease in telling of the sudden deaths that cursed . . .