Peutz-Jeghers syndrome and management recommendations

被引：310

作者：

Giardiello, FM

Trimbath, JD

机构：

[1] Johns Hopkins Univ, Sch Med, Dept Med, Baltimore, MD 21218 USA

[2] Johns Hopkins Univ, Sch Med, Ctr Oncol, Baltimore, MD 21218 USA

[3] Johns Hopkins Univ, Sch Med, Dept Pathol, Baltimore, MD 21218 USA

来源：

CLINICAL GASTROENTEROLOGY AND HEPATOLOGY | 2006年 / 4卷 / 04期

关键词：

D O I：

10.1016/j.cgh.2005.11.005

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

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.

引用

页码：408 / 415

页数：8

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