The Peutz Jeghers Syndrome: A Case Report
نویسنده
چکیده
Peutz Jeghers syndrome ( PJ S ) is an autosomal dominant disease that combines hamartomatous polyposis ,a periorificial lentiginose and a high risk of associated cancers. We report the observation of a girl 07 years old of personal historyof acute intestinal intussusception occurred a year ago who consults for signs of early puberty and the onset of vaginal bleeding up to 06 months . The child has a sexual stage development S3P3R (+) , an advance of height (11 years) and bone age ( 12years) and the presence of brownish macules on the buccal mucosa . During her hospitalization, she developed severe abdominal pain predominant in the right iliac region , with vomiting and a stop materials and gas. The diagnosis of acute intussusception was made and surgical resection of the small intestine is rapidly performed. Histological examination showed a hamartomatous polyp with moderate dysplasia. Endocrine exploration concluded the diagnosis of pseudoprecocious iso sexual puberty related bilateral tumor of the granulosa and digestive endoscopy showed the presence of multiple polyps stepped located at the fundus and small intestine. The girl was treated with cyproterone acetate (for lack of aromatase inhibitors ) and directed to a surgical bilateral oophorectomy because of the high risk of neoplasia. The SPJ can affect many organs with an increased risk of cancers. Obstructive episodes are the main clinical manifestations. We must recognize the syndrome especially if it associate with precocious puberty and / or peri orificial lentiginose
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Unexpected Peutz-Jeghers syndrome in an adult presenting with intermittent upper intestinal obstruction. A case report.
Peutz-Jeghers syndrome is an autosomal dominant inherited disease, belonging to the hamartomatous polyposis syndromes. It is characterized by multiple hamartomatous polyps of the gastrointestinal tract associated with oral and anal mucocutaneous pigmentations. We report the case of an adult patient diagnosed with an atypical form of Peutz-Jeghers syndrome, thereby emphasizing the different poss...
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