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Hamartomatosis polyposis: Peutz-jeghers syndrome: case report and review of the literature


Gastroenterology & Hepatology: Open Access
Bouchrit S Roudi S, B.kouwakanou, Ait Errami A, Oubaha S, Samlani Z, Krati K

Abstract

Peutz-jeghers syndrome is a rare inherited disorder with autosomal dominant transmission that manifests primarily as peri-orificial cutaneous-mucous lentiginosis and gastrointestinal hamartomatous polyps with the possibility of other locations in the lungs and reproductive organs. The main complications are gastrointestinal bleeding, bowel obstruction and rectal prolapse. This disease is associated with a high risk of malignant degeneration not only digestive but also pulmonary, mammary, testicular hence the need for a regular long-term codified monitoring. The treatment is most often conservative especially in children in growth period.

Keywords

Peutz-Jeghers syndrome, hamartomatous polyps, digestive cancers, hamartoma-dysplasia-cancer, ileal loop, chronic abdominal pain, esophagus, stomach, small intestine, colon, rectum, pancreas

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