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“非特異性多発性小腸潰瘍症”は,1966年岡部1)らが,「非特異性原発性小腸潰瘍症の3例」として報告したのを嚆矢とする.その後崎村らと共に症例を重ね2),1970年には“非特異性多発性小腸潰瘍症”として,本症の6例について詳細な臨床的病理学的検討を加え,特に限局性腸炎との異同を中心に詳述している3).
即ち,本症は,臨床的には主として若年者を侵し,腸管からの長年月に亘る潜・顕出血,高度の続発性貧血を主症状とする.病理学的には回腸中~下部の浅い多発性潰瘍または潰瘍瘢痕の形成を主病像とし,限局性腸炎の特徴的所見とされる異常に強い結合織の増殖や,granulomaはみられない.その組織反応の差は,限局性腸炎を“ulcerative proliferative process”と呼べば,本症は“ulcerative non-proliferative process”とでも呼ぶべき疾患であろうと述べている.
Ulcerative, non-proliferative disease of the intestine is clinically seen in young adults, and its cardinal symptoms include persistent occult blood in the stool of many years' duration, marked anemia and hypoproteinemia due to the covert bleeding. Usually it is unaccompanied with diarrhea, fever or malabsorption. Relapse is most common with re-bleeding even after surgical correction, but through its long lasting course no perforation, fistulization, intestinal obstruction or abscess is to be seen. Medical management including steroid hormones fails to alter its course.
Basic macroscopical pictures of ulcerative, nonproliferative disease of the intestine are multiple shallow ulcers developing crosswise or obliquely on the intestinal mucosa. The terminal ileum remains free from ulceration. Its microscopic features are multiple shallow ulcers mostly confined within the submucosa. Accompanying inflammaion is also of slight degree. Features seen in Crohn's disease, such as severe edema, thick proliferation of connective tissue and granuloma, can not be observed.
It is shown here that ulcerative, non-proliferative disease of the intestine differs, both in clinical pictures and in tissue reaction, from Crohn's disease. The present status of the diagnosis of Crohn's disease has also been described, which is at present made solely after exclusion of other specific inflammations of the intestine. We hereby propose that non-specific ulcers of the small intesitne be classified as follows: ―
Non-specific ulcers of the small intestine
1. Due to known causes: enteric coated tablets of KCL, irradiation therapy, etc.
2. Intestinal ulceration as a regional manifestation of systemic diseases: collagen diseases, intestinal Behçet, uremia, etc.
3. Non-specific ulcers of the small intestine in a narrow sense (Primary non-specific ulcers of the small intestine)
1) Crohn's disease
2) Ulcerative, non-proliferative disease of the small intestine
3) Chronic ulcerative jejunoileitis
4) Simple ulcer
5) Others
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