Palabras clave
Niños
Microbiología
Función pulmonar
Genética
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Resumen
Objetivo: Describir los aspectos clínicos, microbiológicos y funcionales de niños y adolescentes atendidos en un servicio de referencia del Nordeste de Brasil. Métodos: Estudio transversal retrospectivo con variables cuantitativas. Resultados: En el estudio participaron 22 pacientes con una edad media de 9 años, de los cuales el 81,8% eran de raza no blanca. La mediana de edad al diagnóstico fue de 6 meses, con predominio de síntomas gastrointestinales y respiratorios crónicos. El cribado neonatal se realizó en el 59% de los niños y la variante F508del se encontró en el 81,8% de los pacientes genotipados. Se recuperaron P. aeruginosa y S. aureaus en el 87,5% y el 57,1% de los pacientes, respectivamente, y la función pulmonar fue normal o con obstrucción leve en el 80% de ellos. Las hospitalizaciones fueron frecuentes en menores de 2 años (58,7%), por exacerbación pulmonar aguda en el 71,5% de los casos y en portadores de la variante F508del. Conclusión: El predominio de genotipos clásicos, el diagnóstico tardío, el bajo número de consultas, el elevado número de hospitalizaciones en los primeros años y la colonización precoz por P. aeruginosa apuntan a la necesidad de mejoras en la red asistencial desde el cribado neonatal, passando por el acceso regular a la medicación, hasta la implementación de políticas públicas que garanticen los derechos de los pacientes.
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