Enfermedad Granulomatosa Crónica, Actualización y Revisión

ITZEL LÓPEZ-HERNÁNDEZ, MARCOS ALEJANDRO SUÁREZ-GUTIÉRREZ, EROS EFRAIN SANTOS-CHÁVEZ, SARA ELVA ESPINOSA-PADILLA, Lizbeth Blancas Galicia

Resumen


La enfermedad granulomatosa crónica (EGC) es un síndrome de inmunodeficiencia primaria caracterizado por una mayor susceptibilidad a desarrollar infecciones fúngicas y bacterianas graves. La EGC es el resultado de una falla de la enzima nicotinamida adenina dinucleotido fosfato (NADPH) oxidasa en los fagocitos del paciente para producir superóxido. Se caracteriza por infecciones recurrentes con un espectro estrecho de bacterias y hongos, así como un conjunto común de complicaciones inflamatorias, entre las que se incluye la enfermedad inflamatoria intestinal. Los patógenos más frecuentemente encontrados son Staphylococcus aureus, especies de Aspergillus, especies de Klebsiella, Burkholderia cepacia, Serratia marcescens y especies de Salmonella. La profilaxis antibiótica a largo plazo ha ayudado a combatir las infecciones asociadas con la enfermedad granulomatosa crónica, mientras que el progreso constante en el trasplante de médula ósea y la posibilidad de la terapia génica son aclamados como opciones de tratamiento permanente.


Palabras clave


inmunodeficiencias primarias; enfermedad granulomatosa crónica; EGC; dihidrorodamina; infecciones recurrentes; defectos de fagocitosis

Referencias


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DOI: http://dx.doi.org/10.29262/ram.v65i1.577

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