Assuntos
Angioedema Hereditário Tipo III/diagnóstico , Edema Laríngeo/diagnóstico por imagem , Anti-Inflamatórios não Esteroides/uso terapêutico , Antifibrinolíticos/uso terapêutico , Bradicinina/análogos & derivados , Bradicinina/uso terapêutico , Criança , Feminino , Angioedema Hereditário Tipo III/complicações , Angioedema Hereditário Tipo III/tratamento farmacológico , Angioedema Hereditário Tipo III/prevenção & controle , Humanos , Edema Laríngeo/etiologia , Peptídeos/uso terapêutico , Tomografia Computadorizada por Raios X , Ácido Tranexâmico/uso terapêuticoRESUMO
Hereditary angioedema (HAE) is a relatively rare clinical entity that can potentially cause life-threatening airway or intestinal oedema, patients with the latter usually presents with symptoms of gastroenteritis like vomiting, diarrhoea and abdominal pain. Here, we present a unique case of a less recognised type of HAE that is type III in a patient who presented with signs and symptoms consistent with infectious colitis. She previously had similar episodes and was managed multiple times with antibiotics, with no satisfactory response. There, she underwent extensive diagnostic evaluation. On the basis of findings of further investigations on the current visit, she was eventually diagnosed with intestinal angioedema. To the best of our knowledge, the present paper represents the third reported case of type III HAE-induced intestinal angioedema. Additionally, we undertake a literature review of HAE.
Assuntos
Colite/diagnóstico , Angioedema Hereditário Tipo III/diagnóstico , Enteropatias/diagnóstico , Infecções Intra-Abdominais/diagnóstico , Diagnóstico Diferencial , Feminino , Angioedema Hereditário Tipo III/complicações , Humanos , Enteropatias/etiologia , Pessoa de Meia-IdadeRESUMO
In type III hereditary angioedema (HAE type III), the phenotype is the same as type I and type II disease, but the level and function of C1-esterase inhibitor (C1-INH) is normal. Hereditary angioedema type III has been described as an oestrogen-sensitive form because it can be triggered or aggravated by exposure to high oestrogen levels as seen during pregnancy, especially when associated with Factor XII mutation. This case report describes the evolution and management of repeated angioedema attacks during pregnancy in a woman with HAE, with normal levels and function of C1-INH (type III); and a mis-sense mutation of factor XII. The physiopathology and genetic features, the unpredictability of clinical manifestations and the management during pregnancy and delivery are discussed.
Assuntos
Proteína Inibidora do Complemento C1/uso terapêutico , Angioedema Hereditário Tipo III/tratamento farmacológico , Angioedema Hereditário Tipo III/fisiopatologia , Complicações Cardiovasculares na Gravidez/tratamento farmacológico , Complicações Cardiovasculares na Gravidez/fisiopatologia , Adulto , Fator XII/genética , Feminino , Angioedema Hereditário Tipo III/complicações , Humanos , Mutação/genética , GravidezRESUMO
Type III hereditary angioedema is a rare familial disorder that has recently been described as a separate condition. Triggers for episodes of angioedema include surgery, dental procedures, and tracheal intubation maneuvers. Since episodes affecting the upper airway are potentially life-threatening, prophylactic treatment is recommended in these situations. The use of icatibant (Firazyr(®)), for prevention of angioedema prior to tracheal intubation, is reported in a patient with type iii hereditary angioedema. A literature review on the anesthetic management of this condition was conducted.