| Theme | Polyposis of the colon and rectum, and other related conditions : hereditary colorectal cancer | |
|---|---|---|
| Title | Juvenile polyposis | |
| Publish Date | 2005/11 | |
| 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. |
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