RESUMEN
Peutz-Jeghers syndrome is an inherited disorder which usually debuts during childhood. It is characterized by mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal tract. Numerous reports indicate a high incidence of gastrointestinal and extraintestinal cancer in these patients, their appearance at a young age, as well as its association with ovarian and testicular tumors. An aggressive approach of these patients seems to be necessary. We report the case of two brothers suffering from Peutz-Jeghers syndrome whose father and grandfather died as a consequence of the progression of an intestinal cancer related to the syndrome.
Asunto(s)
Síndrome de Peutz-Jeghers/diagnóstico , Niño , Diagnóstico Diferencial , Progresión de la Enfermedad , Humanos , Masculino , Melanosis/diagnóstico , Índice de Severidad de la EnfermedadRESUMEN
El síndrome de Peutz-Jeghers es un raro proceso hereditario que suele iniciarse en la infancia. Se caracteriza por la presencia de lesiones cutáneas pigmentadas y pólipos gastrointestinales. Numerosos estudios revelan una incidencia elevada de cáncer (gastrointestinal y extradigestivo) en estos enfermos y su aparición a temprana edad, así como su asociación con tumores ováricos y testiculares. Por ello, es necesario un estrecho seguimiento y un tratamiento agresivo de estos enfermos. Presentamos 2 hermanos afectados de síndrome de Peutz-Jeghers cuyo padre y abuelo fallecieron a consecuencia de cáncer digestivo relacionado con la enfermedad (AU)
Peutz-Jeghers syndrome is an inherited disorder which usually debuts during childhood. It is characterized by mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal tract. Numerous reports indicate a high incidence of gastrointestinal and extraintestinal cancer in these patients, their appearance at a young age, as well as its association with ovarian and testicular tumors. An aggresive approach of these patients seems to be necesary. We report the case of two brothers suffering from Peutz-Jeghers syndrome whose father and grandfather died as a consecuence of the progression of an intestinal cancer related to the síndrome (AU)
Asunto(s)
Humanos , Masculino , Niño , Síndrome de Peutz-Jeghers/diagnóstico , Melanosis/etiología , Pólipos Intestinales/etiología , Dolor Abdominal/etiología , Hemorragia Gastrointestinal/etiologíaRESUMEN
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