INTESTINE Vol.9 No.6(2-3)

特集名 大腸ポリポーシスと関連疾患 (遺伝性大腸癌)
題名 ポリポーシス (3) 若年性ポリポーシス
発刊年月 2005年 11月
著者 石田 秀行 埼玉医科大学総合医療センター消化管・一般外科
著者 岩間 毅夫 佐々木研究所付属杏雲堂病院外科
【 要旨 】 要旨はありません。
Theme Polyposis of the colon and rectum, and other related conditions : hereditary colorectal cancer
Title Juvenile polyposis
Author Hideyuki Ishida Department of Digestive Tract and General Surgery, Saitama Medical Center / School
Author Takeo Iwama Department of Surgery, Kyoundo Hospital, Sasaki Foundation
[ Summary ] Juvenile polyposis (syndrome) (JPS) is an autosomal dominant disorder which is characterized by the presence of juvenile polyps throughout the gastointestinal tract and increased risk of gastrointestinal malignancy. Germline mutations of the SMAD4 gene on chromosome 18q21.1 or the BMPR1A gene on chromosome 10q22-q23 have been found to be causative factors for this disease in a subset of JPS patients.
Clinically, removing polyps in the large intestine is important because these polyps can cause bleeding and intussusception, and can also develop into cancer. Gastrectomy or (procto) colectomy is preferred when innumerable polyps are present in the stomach or large intestine, because these lesions can cause protein-losing gastroenteropathy or can be a focus of cancer.
In this article, three patients with JPS in a kindred who have been followed-up on over an extended period are also presented.