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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Main Authors: Carlos Enríquez Fernández Montoya, Zhachel Alejandro Redondo Gómez, Dayné García García
Format: Article
Language:Spanish
Published: Centro Nacional de Información de Ciencias Médicas. Editorial de Ciencias Médicas (ECIMED) 2022-11-01
Series:Revista Cubana de Anestesiología y Reanimación
Subjects:
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.
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publisher Centro Nacional de Información de Ciencias Médicas. Editorial de Ciencias Médicas (ECIMED)
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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
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AT daynegarciagarcia anesthesiainapatientwithhereditaryangioedema