Anesthesia in a patient with hereditary angioedema
Hereditary angioedema (HAE) is a rare genetic disease caused by an autosomal dominant mutation that results in an alteration of the gene encoding the activated C1 esterase inhibitor protein (C1-INH), causing deficiency or dysfunction of C1-INH. It is characterized by recurrent and self-limited episo...
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Format: | Article |
Language: | Spanish |
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Centro Nacional de Información de Ciencias Médicas. Editorial de Ciencias Médicas (ECIMED)
2022-11-01
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Series: | Revista Cubana de Anestesiología y Reanimación |
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Online Access: | https://revanestesia.sld.cu/index.php/anestRean/article/view/828 |
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author | Carlos Enríquez Fernández Montoya Zhachel Alejandro Redondo Gómez Dayné García García |
author_facet | Carlos Enríquez Fernández Montoya Zhachel Alejandro Redondo Gómez Dayné García García |
author_sort | Carlos Enríquez Fernández Montoya |
collection | DOAJ |
description | Hereditary angioedema (HAE) is a rare genetic disease caused by an autosomal dominant mutation that results in an alteration of the gene encoding the activated C1 esterase inhibitor protein (C1-INH), causing deficiency or dysfunction of C1-INH. It is characterized by recurrent and self-limited episodes with transient symptoms of swelling without urticaria of subcutaneous tissues, extremities, intestinal wall, genitalia and upper respiratory tract. Involvement of the larynx and glottis may result in death by asphyxia. The perioperative managment is reported of a patient with HAE and a long history of allergies in which the main considerations are related to the prevention of an acute crisis during the perioperative period. This required a preparation with fresh frozen plasma and tranexamic acid days before surgery, which was continued postoperatively, in addition to careful management during the anesthetic procedure. |
first_indexed | 2024-04-10T22:33:22Z |
format | Article |
id | doaj.art-d9b905e01f584fa4b115e355213194d3 |
institution | Directory Open Access Journal |
issn | 1726-6718 |
language | Spanish |
last_indexed | 2024-04-10T22:33:22Z |
publishDate | 2022-11-01 |
publisher | Centro Nacional de Información de Ciencias Médicas. Editorial de Ciencias Médicas (ECIMED) |
record_format | Article |
series | Revista Cubana de Anestesiología y Reanimación |
spelling | doaj.art-d9b905e01f584fa4b115e355213194d32023-01-16T22:00:25ZspaCentro Nacional de Información de Ciencias Médicas. Editorial de Ciencias Médicas (ECIMED)Revista Cubana de Anestesiología y Reanimación1726-67182022-11-01213701Anesthesia in a patient with hereditary angioedemaCarlos Enríquez Fernández Montoya0Zhachel Alejandro Redondo Gómez1Dayné García García2Hospital "Dr. Gustavo Aldeguería Lima"Instituto Nacional de Oncología y RadiobiologíaInstituto Nacional de Oncología y RadiobiologíaHereditary angioedema (HAE) is a rare genetic disease caused by an autosomal dominant mutation that results in an alteration of the gene encoding the activated C1 esterase inhibitor protein (C1-INH), causing deficiency or dysfunction of C1-INH. It is characterized by recurrent and self-limited episodes with transient symptoms of swelling without urticaria of subcutaneous tissues, extremities, intestinal wall, genitalia and upper respiratory tract. Involvement of the larynx and glottis may result in death by asphyxia. The perioperative managment is reported of a patient with HAE and a long history of allergies in which the main considerations are related to the prevention of an acute crisis during the perioperative period. This required a preparation with fresh frozen plasma and tranexamic acid days before surgery, which was continued postoperatively, in addition to careful management during the anesthetic procedure.https://revanestesia.sld.cu/index.php/anestRean/article/view/828angioedema hereditariodeficiencia de c1-inhanestesia |
spellingShingle | Carlos Enríquez Fernández Montoya Zhachel Alejandro Redondo Gómez Dayné García García Anesthesia in a patient with hereditary angioedema Revista Cubana de Anestesiología y Reanimación angioedema hereditario deficiencia de c1-inh anestesia |
title | Anesthesia in a patient with hereditary angioedema |
title_full | Anesthesia in a patient with hereditary angioedema |
title_fullStr | Anesthesia in a patient with hereditary angioedema |
title_full_unstemmed | Anesthesia in a patient with hereditary angioedema |
title_short | Anesthesia in a patient with hereditary angioedema |
title_sort | anesthesia in a patient with hereditary angioedema |
topic | angioedema hereditario deficiencia de c1-inh anestesia |
url | https://revanestesia.sld.cu/index.php/anestRean/article/view/828 |
work_keys_str_mv | AT carlosenriquezfernandezmontoya anesthesiainapatientwithhereditaryangioedema AT zhachelalejandroredondogomez anesthesiainapatientwithhereditaryangioedema AT daynegarciagarcia anesthesiainapatientwithhereditaryangioedema |