Enfermedad por micobacterias en pacientes con enfermedad granulomatosa crónica

Autores/as

DOI:

https://doi.org/10.29262/ram.v658i2.859

Palabras clave:

granulomatosa crónica, Bacillus de Calmette-Guérin, tuberculosis, BCGitis, BCGosis, Infección micobacteriana, micobacterias atípicas.

Resumen

La enfermedad granulomatosa crónica (ECG) es un error innato de la inmunidad que afecta la funcionalidad de la fagocitosis, específicamente hay una falta de producción de radicales libres de oxígeno por la NADPH oxidasa. La EGC se manifiesta con infecciones bacterianas y fúngicas, recurrentes y graves e hiperinflamación local y sistémica. En países en donde la tuberculosis es endémica y la vacuna BCG es obligatoria al nacer, los pacientes con EGC pueden presentar como primera manifestación las reacciones locales o sistémicas a esta vacuna y además a lo largo de su vida infecciones recurrentes por M. tuberculosis. La susceptibilidad de estos pacientes a micobacterias es debida a la producción excesiva de citocinas proinflamatorias y la formación de granulomas ineficientes en la contención de la micobacteria. En los países desarrollados, los pacientes con EGC no presentan este tipo de manifestaciones infecciosas, salvo los migrantes de países en desarrollo. En esta revisión, presentamos las características de las infecciones por BCG, M. tuberculosis y otras micobacterias. Interesantemente no existen lineamientos en cuanto a los tratamientos antituberculosos en pacientes con EGC, por lo que proponemos realizar un consenso por expertos para establecer lineamientos para el tratamiento de la enfermedad por micobacterias en EGC.

Biografía del autor/a

Lizbeth Blancas-Galicia, Secretaría de Salud, Instituto Nacional de Pediatría, Unidad de Investigación en Inmunodeficiencias, Ciudad de México

La incidencia de la enfermedad granulomatosa crónica (EGC) en reportes internacionales es de 1:250, 000, sin embargo en México se desconoce. En el Instituto Nacional de Pediatría a partir del 2009 se implementó un proyecto para facilitar diagnóstico de dicha enfermedad. De esa fecha al día de hoy se han estudiado 67 casos, de los cuales el 80% son formas ligadas al X (LX), además de que cada vez mas han se diagnosticado los casos a una edad más temprana. Actualmente nos encontramos con un nuevo reto, el tratamiento curativo de los pacientes con EGC. Presentamos el caso de un paciente con EGC-LX trasplantado exitosamente al mes de vida y trasplantado de forma exitosa en México.

Citas

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Publicado

2021-08-22

Número

Sección

Inmunología