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Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report -

Anesthesia and Pain Medicine 2021년 16권 1호 p.75 ~ 80
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Abstract


Background: 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).

Case: 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.

Conclusions: The anesthesiologist should prepare for the critical complications of HAE and prepare the appropriate treatment options.

키워드

Capillary leak syndrome; Complement C1 inhibitor protein; Complement C1s; Disseminated intravascular coagulation; Hereditary angioedema; Kidney transplantation

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