Angioedema hereditario en Costa Rica

Alberto Josué Alfaro Murillo, Mario Martínez Alfonso, José Castro Cordero, María Paz León Bratti, Gabriela Ivankovich Escoto, María del Rosario Espinoza Mora

Resumen


Resumen

 

Antecedentes: El angioedema hereditario se encuentra clasificado como una inmunodeficiencia primaria del sistema de complemento, debido a que se caracteriza por la ausencia de C1 inhibidor esterasa y por edema periódico de cualquier región del cuerpo que involucre tejido blando.

Objetivo: Caracterizar a los pacientes adultos con diagnóstico de angioedema hereditario atendidos en el servicio de alergología clínica del Hospital México de la Caja Costarricense del Seguro Social.

Métodos: El estudio realizado fue observacional retrospectivo. Los datos fueron obtenidos de los expedientes clínicos de todos los pacientes con diagnóstico confirmado de angioedema hereditario que estaban en seguimiento en el Servicio de Alergología del Hospital México.

Resultados: Se documentó un total de 14 pacientes, 7 hombres y 7 mujeres. El promedio de edad encontrado fue de 36,6 años. Las manifestaciones clínicas más frecuentes fueron edema de extremidades y dolor abdominal. Solo 3 pacientes en algún momento durante su evolución presentaron edema laríngeo. 12 de los casos correspondieron con angioedema  hereditario tipo I.

Conclusiones: El comportamiento clínico de todos los casos documentados correspondió con el descrito para la patología, aunque solamente pocos pacientes contaban con historial de manifestaciones graves. 

 

Abstract

 

Background: Hereditary angioedema is classified as a primary complement immunodeficiency. It is characterized by the absence of C1 esterase inhibitor (C1-INH) and by periodic edema of any region of the body that involves soft tissue. 

Objective: Characterize the adult patients with confirmed diagnosis of hereditary angioedema treated in the Clinical Allergology Department of the Hospital México, Caja Costarricense del Seguro Social. 

Methods: The study was retrospective and observational. All clinical records of patients with hereditary angioedema controlled in the Allergology Service of the Hospital México were reviewed.

Results: A total of 14 patients, 7 men and 7 women were documented. The average age found was 36.6 years. The most frequent clinical manifestations were limb edema and abdominal pain. Only 3 patients at some time during their evolution presented laryngeal edema. 12 cases corresponded to hereditary angioedema type I.

Conclusions: The clinical behavior of all documented cases corresponded to that described for the pathology, although only a few patients had a history of serious manifestations.



Palabras clave


angioedema; proteína inhibitoria C1 del complemento; SERPING1; bradiquinina; calicreína.

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DOI: http://dx.doi.org/10.29262/ram.v67i1.722

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