Park Jeong Wook, Seo Jinyoung, Kim Sang Hun, Jung Ki Tae
Department of Anesthesiology and Pain Medicine, Chosun University Hospital, Gwangju, Korea.
Department of Medicine, Graduate School, Chosun University, Gwangju, Korea.
Anesth Pain Med (Seoul). 2021 Jan;16(1):75-80. doi: 10.17085/apm.20098. Epub 2021 Jan 27.
Hereditary angioedema (HAE) is a rare disease caused by the deficiency of C1 esterase inhibitor. HAE has a risk of life-threatening complications such as capillary leak syndrome (CLS) and disseminated intravascular coagulation (DIC).
A 42-year-old male patient with HAE presented for deceased-donor kidney transplantation. Prophylactic fresh frozen plasma (FFP) was given before surgery because of the risk of edema development. With careful management during anesthesia, there were no problems during surgery. However, generalized edema, hypotension, hypoalbuminemia, massive drainage of serosanguineous fluids from the intraabdominal space, and DIC occurred on the day after surgery. CLS was suspected and sustained hypotension with generalized edema became worse despite treatment with albumin, danazol, FFP, and vasoactive drugs. The patient's condition worsened despite intensive care and he died due to shock.
The anesthesiologist should prepare for the critical complications of HAE and prepare the appropriate treatment options.
遗传性血管性水肿（HAE）是一种由C1酯酶抑制剂缺乏引起的罕见疾病。HAE存在危及生命的并发症风险，如毛细血管渗漏综合征（CLS）和弥散性血管内凝血（DIC）。
一名42岁的HAE男性患者接受已故供体肾移植。由于有发生水肿的风险，术前给予了预防性新鲜冰冻血浆（FFP）。在麻醉期间进行了精心管理，手术过程中未出现问题。然而，术后第一天出现了全身性水肿、低血压、低白蛋白血症、腹腔内大量血性液体引流以及DIC。怀疑发生了CLS，尽管使用了白蛋白、达那唑、FFP和血管活性药物治疗，但全身性水肿伴持续性低血压仍加重。尽管进行了重症监护，患者的病情仍恶化，最终因休克死亡。
麻醉医生应做好应对HAE严重并发症的准备，并准备好适当的治疗方案。
