Japanese Clinical Guidelines 2020 for Diagnosis and Treatment of Peutz-Jeghers Syndrome in Children and Adults
-
- Yamamoto Hironori
- Department of Medicine, Division of Gastroenterology, Jichi Medical University
-
- Abe Takashi
- Department of Gastroenterology, Hanwa Sumiyoshi General Hospital
-
- Ishiguro Shingo
- PCL JAPAN, INC
-
- Uchida Keiichi
- Department of Pediatric Surgery, Mie University Hospital
-
- Kawasaki Yuko
- University of Hyogo, College of Nursing
-
- Kumagai Hideki
- Department of Pediatrics, Jichi Medical University
-
- Saida Yoshihisa
- Department of Surgery, Toho University Ohashi Medical Center
-
- Sano Yasushi
- Gastrointestinal Center & Institute of Minimallyinvasive Endoscopic Care, Sano Hospital
-
- Takeuchi Yoji
- Department of Gastrointestinal Oncology, Osaka International Cancer Institute
-
- Tajika Masahiro
- Depertment of Endoscopy, Aichi Cancer Center
-
- Nakajima Takeshi
- Clinical Genetics, Cancer Institute Hospital
-
- Banno Kouji
- Departmint of Obstetrics and Gynecology, Keio University School of Medicine
-
- Funasaka Yoko
- Department of Dermatology, Nippon Medical School
-
- Hori Shinichiro
- Department of Cancer Genomic Medicine, NHO Shikoku Cancer Center
-
- Yamaguchi Tatsuro
- Department of Surgery and Department of Clinical Genetics, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital
-
- Yoshida Teruhiko
- Department of Genetic Medicine and Service, National Cancer Center Hospital
-
- Sakamoto Hirotsugu
- Department of Medicine, Division of Gastroenterology, Jichi Medical University
-
- Ishikawa Hideki
- Department of Molecular-Targeting Prevention, Kyoto Prefectural University of Medicine・Ishikawa Gastroenterology Clinic
-
- Iwama Takeo
- Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
-
- Okazaki Yasushi
- Intractable Disease Research Center, Graduate School of Medicine, Juntendo University
-
- Saito Yutaka
- Endoscopy Division, National Cancer Center Hospital
-
- Matsuura Nariaki
- Osaka International Cancer Institute
-
- Mutoh Michihiro
- Department of Molecular-Targeting Prevention, Graduate School of Medical Science, Kyoto Prefectural University of Medicine
-
- Tomita Naohiro
- Cancer Treatment Center, Toyonaka Municipal Hospital
-
- Akiyama Takashi
- Department of Pediatric Surgery, Chuden Hospital
-
- Yamamoto Toshiki
- Division of Gastroenterology and Hepatology, Department of Medicine, Nihon University School of Medicine
-
- Ishida Hideyuki
- Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University
-
- Nakayama Yoshiko
- Department of Pediatrics, Shinshu University School of Medicine
Bibliographic Information
- Other Title
-
- 小児・成人のためのPeutz-Jeghers 症候群診療ガイドライン（2020 年版）
Search this article
Abstract
Peutz-Jeghers syndrome (PJS) is a rare disease characterized by the presence of hamartomatous polyposis throughout the gastrointestinal tract, except for the esophagus, along with characteristic mucocutaneous pigmentation. It is caused by germline pathogenic variants of the STK11 gene that exhibits an autosomal dominant mode of inheritance. PJS may be identified by multigene panel testing in patients with cancer. In this syndrome, there is persistent development of hamartomatous polyps in the small bowel. Such polyps may cause bleeding, intestinal obstruction, and intussusception as they grow larger. The initial gastrointestinal surveillance should be performed at about eight years of age even if no symptoms are present. Endoscopic polypectomy should be performed for small bowel polyps with a diameter of 10-15 mm or larger. Appropriate surveillance is required because patients with this syndrome may develop malignant tumors of different organs such as the digestive tracts, breasts, pancreas, uterus, ovaries, lungs, and testes. The present clinical guidelines explain the principles in the diagnosis and management of PJS, together with four clinical questions and corresponding recommendations. The guidelines have been designed to promote seamless implementation of accurate diagnosis and appropriate management of pediatric, adolescent and adult patients with the disease.
Journal
-
- Journal of Hereditary Tumors
-
Journal of Hereditary Tumors 20 (2), 59-78, 2020
The Japanese Society for Hereditary Tumors
- Tweet
Details 詳細情報について
-
- CRID
- 1390848647553001600
-
- NII Article ID
- 130007908484
-
- ISSN
- 24356808
-
- Text Lang
- ja
-
- Data Source
-
- JaLC
- CiNii Articles
- KAKEN
-
- Abstract License Flag
- Disallowed