Peutz Jeghers syndrome (gastro-intestinal polyposis) and its complications.
نویسندگان
چکیده
A rare case of multiple jejunal poiyps, presenting as an acute abdominal emergency with complications of mtussusception and gangrene is discussed. The relevant features of Peutz Jegher’s syndrome and the surgical management of it’s complications are highlighted (JPMA 35 : 154, 1985).
منابع مشابه
Colonic adenocarcinoma and bilateral malignant ovarian sex cord tumor with annular tubules in Peutz-Jeghers syndrome.
Peutz-Jeghers syndrome is characterized by multiple polyps throughout the gastrointestinal tract in association with mucocutaneous pigmentation. Although Peutz-Jeghers syndrome polyps are hamartomas, frequent association of this syndrome with both gastrointestinal and non-gastrointestinal tumours had led to reassessment of the cancer risk in this hereditary disorder. The most common gynaecologi...
متن کاملPeutz-Jeghers syndrome
Introduction Peutz-Jeghers syndrome (PJS) is characterized by: (i) autosomal dominant inheritance; (ii) cutaneous pigmentation; (iii) gastro-intestinal polyposis. In all, more than 300 cases have been described with a world-wide distribution and no racial predilection. In 1921 Peutz described 7 cases of multiple intestinal polyps associated with melanin spots on the lips, buccal mucosa and digi...
متن کاملPeutz-Jeghers syndrome: a new understanding.
Peutz-Jeghers syndrome is an autosomal dominant inherited disorder characterized by hamartomatous polyps in the small bowel and mucocutaneous pigmentation. Patients with Peutz-Jeghers syndrome often present as surgical emergencies with complications of the polyps, such as intussusception, bowel obstruction and bleeding. Furthermore, repeated operations may be needed in some patients, which may ...
متن کاملPeutz-Jeghers syndrome, case report.
Peutz-Jeghers syndrome (PJS) is an unusual hamartomatous polyposis of the gastro intestinal (GI) tract, with pigmentation around lips and macules on the buccal mucosa. The case of a 10-year-old girl who presented with intussusception is reported. A polyp was found to be the cause of an invagination. Histologically it was a hamartoma. PJS is a rare syndrome inherited in an autosomal dominant pat...
متن کاملKrukenberg tumour as the initial presentation of Peutz-Jeghers syndrome
Peutz-Jeghers syndrome (PJS) is an hereditary syndrome characterized by gastrointestinal polyposis and mucocutaneous pigmentation. PJS patients are at increased risk of developing various cancers, especially of the gastrointestinal and gynaecological tracts. Colonic adenocarcinoma is one of the more common tumours that occur in PJS. We report a young lady presenting with a large ovarian tumour,...
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ورودعنوان ژورنال:
- JPMA. The Journal of the Pakistan Medical Association
دوره 35 5 شماره
صفحات -
تاریخ انتشار 1985