Neurofibromatosis tipo 1 y sangrado de intestino delgado. Reporte de caso
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Gómez-Zuleta, Martín Alonso
Lúquez-Mindiola, Adán José
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EspañolFecha de publicación
2017-10-01
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Resumen
La neurofibromatosis tipo 1, o enfermedad de von Recklinghausen, es un desorden neurocutáneo hereditario con compromiso gastrointestinal en el 5-25% de los pacientes, siendo sintomático solo el 5%; se presenta posterior a las manifestaciones cutáneas y afecta en su mayoría el yeyuno. Los síntomas de esta enfermedad son dolor abdominal, obstrucción intestinal, perforación, diarrea, masa palpable y sangrado gastrointestinal alto o bajo.A continuación se reporta el caso de una mujer con sangrado gastrointestinal manifiesto secundario a neurofibromas plexiformes en yeyuno y se realiza una breve revisión de la literatura sobre compromiso gastrointestinal.
Neurofibromatosis type 1, also known as von Recklinghausen disease, is an inherited neurocutaneous disorder with gastrointestinal involvement in 5-25% of patients, with only 5% being symptomatic; it develops following cutaneous manifestations and mostly affects the jejunum. The symptoms include abdominal pain, intestinal obstruction, perforation, diarrhea, palpable mass and high or low gastrointestinal bleeding.The following report presents the case of a woman with manifest gastrointestinal bleeding secondary to plexiform neurofibromas in the jejunum, as well as a brief review of the literature on gastrointestinal involvement.
Neurofibromatosis type 1, also known as von Recklinghausen disease, is an inherited neurocutaneous disorder with gastrointestinal involvement in 5-25% of patients, with only 5% being symptomatic; it develops following cutaneous manifestations and mostly affects the jejunum. The symptoms include abdominal pain, intestinal obstruction, perforation, diarrhea, palpable mass and high or low gastrointestinal bleeding.The following report presents the case of a woman with manifest gastrointestinal bleeding secondary to plexiform neurofibromas in the jejunum, as well as a brief review of the literature on gastrointestinal involvement.