Palabras clave
Enfermedad granulomatosa crónica
Dihidrorodamina
Infecciones recurrentes
Defectos de fagocitosis
Resumen
La enfermedad granulomatosa crónica es un síndrome de inmunodeficiencia primaria caracterizado por mayor susceptibilidad para desarrollar infecciones fúngicas y bacterianas graves. La enfermedad granulomatosa crónica es el resultado de una falla de la enzima nicotinamida adenina dinucleótido fosfato oxidasa en los fagocitos del paciente para producir superóxido. Se caracteriza por infecciones recurrentes con un espectro estrecho de bacterias y hongos, así como por un conjunto común de complicaciones inflamatorias, entre las que se incluye la enfermedad inflamatoria intestinal. Los patógenos más frecuentemente encontrados son Staphylococcus aureus, Aspergillus spp., Klebsiella spp., Burkholderia cepacia, Serratia marcescens y Salmonella spp. 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 ser perfilan como opciones de tratamiento permanente.
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