Peutz-Jeghers Syndrome and Management Recommendations

Francis M. Giardiello; Jill D. Trimbath

doi:10.1016/j.cgh.2005.11.005

Peutz-Jeghers Syndrome and Management Recommendations

Francis M. Giardiello, Jill D. Trimbath

School of Medicine

Research output: Contribution to journal › Review article › peer-review

Abstract

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disease caused by germline mutation of the serine threonine kinase 11 and characterized by hamartomatous polyps in the gastrointestinal tract and mucocutaneous melanin pigmentation. Patients with PJS are at increased risk for common and unusual types of gastrointestinal and nongastrointestinal tumors. This review analyzes currently available literature and describes the clinical characteristics of PJS, assesses the risk of malignancy in this disorder, and delineates management and surveillance recommendations for affected individuals.

Original language	English (US)
Pages (from-to)	408-415
Number of pages	8
Journal	Clinical Gastroenterology and Hepatology
Volume	4
Issue number	4
DOIs	https://doi.org/10.1016/j.cgh.2005.11.005
State	Published - Apr 2006

ASJC Scopus subject areas

Hepatology
Gastroenterology

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AB - Peutz-Jeghers syndrome (PJS) is an autosomal dominant disease caused by germline mutation of the serine threonine kinase 11 and characterized by hamartomatous polyps in the gastrointestinal tract and mucocutaneous melanin pigmentation. Patients with PJS are at increased risk for common and unusual types of gastrointestinal and nongastrointestinal tumors. This review analyzes currently available literature and describes the clinical characteristics of PJS, assesses the risk of malignancy in this disorder, and delineates management and surveillance recommendations for affected individuals.

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Peutz-Jeghers Syndrome and Management Recommendations

Abstract

ASJC Scopus subject areas

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