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家族性大腸ポリープ症は大腸全域にびまん性に無数の腺腫性ポリープを発生し,放置すれば高率に癌化を来す家族性発生が濃厚な疾患である.1882年Cripps1)の兄妹2例の報告以来,その発生機序および癌化の点で興味ある疾患として注目され,最近諸家の報告も散見されるようになった.諸外国,とくに英米両国においては長年月にわたる観察例を有し,本邦においても宇都宮ら2)が394例余の全国集計例を報告している.
著者らは27歳,一卵性双生児の男子2例に本症の発生をみ,明らかな癌性変化を認めたが,術後3年余経過した現在再発なく正常に社会復帰している症例を経験したので,若干の文献的考察を加えて報告する.
Case 1
A 27-year-old male visited our Hospital complaining of bloody stool for the past four years. Following a diagnosis of polyposis of the colon on the basis of X-ray and endoscopic findings and of the histology of biopsy specimen from the rectum, the patient underwent a total colectomy with ileorectal anastomosis.
In the resected specimen, as many as 1,631 polypoid lesions were revealed throughout the colon, with their size ranging from a rice-grain to a finger-tip. One of the polyps of the sigmoid colon showed malignant changes.
Case 2
Based on this experience, the counterpart of the twin was examined and found to have the identical findings as found in his brother ; he received the same surgical treatment. The resected specimen disclosed 1,954 polypoid lesions with malignant changes in lesions of the cecum and sigmoid colon.
The patients have had an uneventful postoperative course with relatively regular bowel movements 3~4 times a day, regaining their weights before the operation and enjoying their daily social lives.
They have been on a close follow-up study with a regular biopsy of remaining polyps in the rectum at every 6 months' interval. These particular patients should be regarded both as a solitary case on the basis of heredity. For these patients, a long-term follow-up study on polypoid lesions of the rectum are necessary.
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