Management of the Peutz-Jeghers Patient

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Medical management update: Peutz Jeghers syndrome.

Peutz Jeghers syndrome (PJS) is an autosomal dominant disease characterized by hamartomatous polyposis and distinct mucocutaneous pigmentation. PJS is associated with an increased risk for several cancers and other complications such as small intestine intussusception, short bowel syndrome, and anemia. Medical management mainly consists of treatment of the polyps and surveillance. This medical ...

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Peutz-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...

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Total gastrointestinal endoscopy in the management of Peutz-Jeghers syndrome.

Peutz-Jeghers syndrome was diagnosed in a 51-year-old woman presenting with iron deficiency anaemia. Upper gastrointestinal endoscopy and colonoscopy revealed several hamartomatous polyps in the stomach, duodenum and colon, which were removed. At a combined surgical-endoscopic procedure, 42 hamartomatous polyps were removed from the small intestine by snare polypectomy. This enteroscopic proced...

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[Peutz-Jeghers syndrome].

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....

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[Peutz-Jeghers syndrome].

Peutz-Jeghers syndrome (PJS, MIM 175,2000) is a disease of autosomal dominant inheritance that is characterised by hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. In addition to problems such as intussusception, PJS predisposes to cancers of several sites. The unusual combination of clinical features makes the identification of the defect underlying PJS particularly intere...

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ژورنال

عنوان ژورنال: Journal of the Royal Society of Medicine

سال: 1989

ISSN: 0141-0768,1758-1095

DOI: 10.1177/014107688908201116