Publicação
Hereditary Angioedema: The Importance of Clinical Suspicion and Proper Treatment
| Resumo: | Hereditary angioedema (HAE) is a syndrome characterizedby episodic swelling of subcutaneous and submucosal tissues, due to a deficiency or dysfunctionality in functionalC1 esterase inhibitor (C1-inh). The authors present a caseof a 40 year-old man with a positive family history of HAE,recurrent episodes of abdominal pain, nausea and vomiting.The immunological study revealed low levels of C1-inh andC4 and normal levels of C1q. Acquired angioedema wasexcluded, making the diagnosis of type 1 HAE. The patientpresented several times to the emergency department withsevere abdominal pain, diffuse abdominal tenderness withguarding. However, laboratory and imagiologic studies hadno alterations. A subcutaneous injection of icatibant was administered with resolution of symptoms. HAE is a rare andpotentially fatal disease, mainly during acute attacks. An early diagnosis and a proper treatment are fundamental. |
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| Autores principais: | Araújo, Ana |
| Outros Autores: | Pinto, Joel; Almeida, Paulo; Pinheiro, Beatriz; Morete, Ana |
| Assunto: | Angioedema Hereditário Icatibant Proteína Inibidora do Complemento C1 Angioedemas, Hereditary Complement C1 Inhibitor Protein Icatibant |
| Ano: | 2018 |
| País: | Portugal |
| Tipo de documento: | artigo |
| Tipo de acesso: | unknown |
| Instituição associada: | Sociedade Portuguesa de Medicina Interna |
| Idioma: | português |
| Origem: | Revista Portuguesa de Medicina Interna |
| Resumo: | Hereditary angioedema (HAE) is a syndrome characterizedby episodic swelling of subcutaneous and submucosal tissues, due to a deficiency or dysfunctionality in functionalC1 esterase inhibitor (C1-inh). The authors present a caseof a 40 year-old man with a positive family history of HAE,recurrent episodes of abdominal pain, nausea and vomiting.The immunological study revealed low levels of C1-inh andC4 and normal levels of C1q. Acquired angioedema wasexcluded, making the diagnosis of type 1 HAE. The patientpresented several times to the emergency department withsevere abdominal pain, diffuse abdominal tenderness withguarding. However, laboratory and imagiologic studies hadno alterations. A subcutaneous injection of icatibant was administered with resolution of symptoms. HAE is a rare andpotentially fatal disease, mainly during acute attacks. An early diagnosis and a proper treatment are fundamental. |
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