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若年性ポリポージスは,胃・小腸・大腸に散在性にみられる有茎・無茎のポリポージスで組織学的に過誤腫として知られている.われわれは1975年にその1例を経験し,武藤らと共に本邦第2例目の症例として報告した5).本症についての認識は広まりつつあるが,なお未解決の点が多い.第1は遺伝形式が明らかでないこと,第2はjuvenilepolyposis coli(以下JPCと省略)とgeneralized juvenile gastrointestinal polyposis(以下GJGPと省略)とが異なる疾患と考えるか否かであり,第3は本症と腺腫症との関係が病理学的にも遺伝学的にも論じられている点であり,第4は本症に発生異常を合併することである.
われわれは18歳男子にみられた直腸に限局した若年性ポリポージスの1例と,28歳女子にみられた胃・結腸の若年性ポリポージス(一部に腺腫が混在した)の1例を経験したので若干の文献的考察を加え報告する.
Two different types of juvenile polyposis coli were presented: juvenile polyposis confined in the rectum and that of stomach and colon with adenomatous pattern.
The first patient was a 15-year-old boy who visited us with persisting rectal bleeding for three months. Multiple polyps were detected in the rectum. Since the symptom could not be controlled by sigmoid scopic polypectomy. Miles operation was performed at the age of 18. The resected specimen clearly showed numerous polyps which accumulated in an area 5 cm in length at the lowest part of the rectum. Histologically, they were typical juvenile polyps.
No adenomatous nor malignant area was seen. No polypoid lesion was detected in the other area of the large bowel nor in the upper gastrointestinal tract. His family history was negative.
The second patient was a 28-year-old woman who visited us in July 1977 complaining of right lower abdominal pain. Barium enema revealed the ileocolic intussusception and polypoid lesions in the caecum. Endoscopic examinations revealed several scattered sessile or pedunculated polyps in the colon and rectum, and polyposis in the stomach. Operative polypectomy at the caecum and appendectomy were performed. The caecal polyp was semipedunculated and of 3×3×3 cm in size; histologically, it was partly adenomatous mainly in juvenile type character. The mucosa of the appendix showed histological appearance represented by the juvenile polyp. Two polyps removed by colonofiberscope showed the histology of juvenile polyp with partly adenomatous changes. Partial gastrectomy was performed in February 1978 because of persisting hypoproteinemia and anemia. The resected specimen revealed extensive polyposis of the antrum which were histologically juvenile polyps. Family history was negative. Heterogeneity in juvenile polyposis coli and the evidence of adenomatous degeneration in such a condition were discussed.
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