Article ; Online: C1-esterase inhibitor for short-term prophylaxis in a patient with hereditary angioedema with normal C1 inhibitor function.
Journal of clinical anesthesia
2016 Volume 35, Page(s) 488–491
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 ... ...
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. |
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MeSH term(s) | Adult ; Angioedemas, Hereditary/complications ; Angioedemas, Hereditary/surgery ; Antifibrinolytic Agents/therapeutic use ; Complement C1 Inactivator Proteins/physiology ; Complement C1 Inhibitor Protein/therapeutic use ; Complement Inactivating Agents/therapeutic use ; Female ; Humans ; Postoperative Complications/prevention & control ; Preoperative Care/methods ; Thyroidectomy ; Tranexamic Acid/therapeutic use |
Chemical Substances | Antifibrinolytic Agents ; Complement C1 Inactivator Proteins ; Complement C1 Inhibitor Protein ; Complement Inactivating Agents ; SERPING1 protein, human ; Tranexamic Acid (6T84R30KC1) |
Language | English |
Publishing date | 2016-12 |
Publishing country | United States |
Document type | Case Reports ; Journal Article |
ZDB-ID | 1011618-7 |
ISSN | 1873-4529 ; 0952-8180 |
ISSN (online) | 1873-4529 |
ISSN | 0952-8180 |
DOI | 10.1016/j.jclinane.2016.08.042 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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