[Prophylaxis in hereditary angioedema with normal C1 inhibitor].

Abstract

Introduction: Hereditary angioedema is an infrequent genetic disorder; which mainly manifests with cutaneous and mucosal swelling. Minor trauma may trigger potentially life-threatening events. In type I and II hereditary angioedema, plasma-derived C1-inhibitor concentrate can be used as short-term prophylaxis. For hereditary angioedema, prophylaxis is not yet standardized, but normal C1 inhibitor could be beneficial.

Case report: A 69-year-old woman, with a genetic diagnosis of hereditary angioedema with normal C1 inhibitor, who needed multiple dental extractions. The surgical procedure was performed under general anesthesia, using 1000 U of plasma-derived C1-inhibitor concentrate as prophylaxis an hour before. The patient was admitted in the ICU for postsurgical care and the outcome was good.

Conclusion: We highlight the possibility of successfully using plasma-derived C1-inhibitor concentrate as prophylaxis in patients with hereditary angioedema with normal C1 inhibitor.