Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report -
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...
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| Format: | Article |
| Language: | English |
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Korean Society of Anesthesiologists
2021-01-01
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| Series: | Anesthesia and Pain Medicine |
| Subjects: | |
| Online Access: | http://www.anesth-pain-med.org/upload/pdf/apm-20098.pdf |
| _version_ | 1797825440422821888 |
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| author | Jeong Wook Park Jinyoung Seo Sang Hun Kim Ki Tae Jung |
| author_facet | Jeong Wook Park Jinyoung Seo Sang Hun Kim Ki Tae Jung |
| author_sort | Jeong Wook Park |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-13T10:53:02Z |
| format | Article |
| id | doaj.art-22d1ed3b5bdd420a8b473c6688e6287b |
| institution | Directory Open Access Journal |
| issn | 1975-5171 2383-7977 |
| language | English |
| last_indexed | 2024-03-13T10:53:02Z |
| publishDate | 2021-01-01 |
| publisher | Korean Society of Anesthesiologists |
| record_format | Article |
| series | Anesthesia and Pain Medicine |
| spelling | doaj.art-22d1ed3b5bdd420a8b473c6688e6287b2023-05-17T05:44:26ZengKorean Society of AnesthesiologistsAnesthesia and Pain Medicine1975-51712383-79772021-01-01161758010.17085/apm.200981061Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report -Jeong Wook Park0Jinyoung Seo1Sang Hun Kim2Ki Tae Jung3 Department of Anesthesiology and Pain Medicine, Chosun University Hospital, Gwangju, Korea Department of Anesthesiology and Pain Medicine, Chosun University Hospital, Gwangju, Korea Department of Anesthesiology and Pain Medicine, Chosun University Hospital, Gwangju, Korea Department of Anesthesiology and Pain Medicine, Chosun University Hospital, Gwangju, KoreaBackground 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.http://www.anesth-pain-med.org/upload/pdf/apm-20098.pdfcapillary leak syndromecomplement c1 inhibitor proteincomplement c1sdisseminated intravascular coagulationhereditary angioedemakidney transplantation |
| spellingShingle | Jeong Wook Park Jinyoung Seo Sang Hun Kim Ki Tae Jung Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report - Anesthesia and Pain Medicine capillary leak syndrome complement c1 inhibitor protein complement c1s disseminated intravascular coagulation hereditary angioedema kidney transplantation |
| title | Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report - |
| title_full | Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report - |
| title_fullStr | Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report - |
| title_full_unstemmed | Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report - |
| title_short | Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report - |
| title_sort | capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema a case report |
| topic | capillary leak syndrome complement c1 inhibitor protein complement c1s disseminated intravascular coagulation hereditary angioedema kidney transplantation |
| url | http://www.anesth-pain-med.org/upload/pdf/apm-20098.pdf |
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