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El querubismo es una enfermedad autosómica dominante de incidencia esporádica, con manifestaciones clínicas progresivas e indoloras de sustitución de tejido óseo normal por tejido fibroso en las regiones mandibular y maxilar. Se presenta el caso de un paciente que consulta a los 3 años de edad, sin antecedentes personales patológicos conocidos, con historia de ausencia parcial de piezas dentales temporales inferiores y aumento de volumen mandibular bilateral, abordado en el Hospital Nacional de Niños Dr. Carlos Sáenz Herrera. Se realiza una revisión sobre los hallazgos clínicos, radiológicos e histopatológicos del paciente, además del tratamiento indicado en este caso, correlacionado con lo descrito en la literatura.
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