title: C1-esterase inhibitor for short-term prophylaxis in a patient with hereditary angioedema with normal C1 inhibitor function.
abstract: Hereditary angioedema with normal C1-esterase inhibitor (HAE-nC1INH) perioperative is a rare condition which could have potential disastrous ramifications for the anesthesiologist in the perioperative period. However, there is limited evidence and/or guidelines on the optimal way to manage these patients. We present the case of a patient with HAE-nC1INH who was successfully managed in the perioperative period with plasma derived C1-esterase inhibitor (pdC1INH). A 29-year-old woman with a diagnosis of HAE-nC1INH presented to the preoperative consultation in preparation for an upcoming total thyroidectomy. She had a 14-year history of ongoing lip and facial edema sometimes necessitating emergency department visitation. Close consultation with her immunologist, transfusion medicine specialists, and anesthesia care providers allowed for a preoperative plan to provide the patient adequate prophylaxis. Both pdC1INH and tranexamic acid were given preoperatively. The patient underwent surgery with no complications. A multidisciplinary team of clinical immunologists, transfusion medicine specialists, and anesthesiologists facilitated the successful perioperative management of a patient with HAE-nC1INH; pdC1INH may a suitable prophylactic perioperative therapy for this rare patient population.
authors.affiliation: Department of Anesthesia, University of Toronto, Toronto, ON M4N 3M5, Canada.
authors.forename: Savio K H
authors.initials: SK
authors.lastname: Yu
authors.affiliation: Department of Clinical Pathology, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON M4N 3M5, Canada; Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON M4N 3M5, Canada.
authors.forename: Jeannie
authors.initials: J
authors.lastname: Callum
authors.affiliation: Department of Anesthesia, Trauma, Emergency and Critical Care Program, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON M4N 3M5, Canada.
authors.forename: Asim
authors.initials: A
authors.lastname: Alam
mesh_terms: D000328:Adult; D054179:Angioedemas, Hereditary / Q000150:complications / Q000601:surgery*; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D003174:Complement C1 Inactivator Proteins / Q000502:physiology; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use*; D051056:Complement Inactivating Agents / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D011183:Postoperative Complications / Q000517:prevention & control*; D011300:Preoperative Care / Q000379:methods; D013965:Thyroidectomy; D014148:Tranexamic Acid / Q000627:therapeutic use
chemical_list: D000933:Antifibrinolytic Agents; D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D051056:Complement Inactivating Agents; C469952:SERPING1 protein, human; D014148:Tranexamic Acid
keywords: HAE-nC1INH; Hereditary angioedema; Normal C1-esterase; Perioperative; Prophylaxis; Type III