TY - JOUR
T1 - Peutz-Jeghers Syndrome and Management Recommendations
AU - Giardiello, Francis M.
AU - Trimbath, Jill D.
PY - 2006/4
Y1 - 2006/4
N2 - 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.
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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U2 - 10.1016/j.cgh.2005.11.005
DO - 10.1016/j.cgh.2005.11.005
M3 - Review article
C2 - 16616343
AN - SCOPUS:33645838599
VL - 4
SP - 408
EP - 415
JO - Clinical Gastroenterology and Hepatology
JF - Clinical Gastroenterology and Hepatology
SN - 1542-3565
IS - 4
ER -