肥厚性皮膚骨膜症を合併し長期にわたって診療中のCEASの1例

Long-term Treatment of Chronic Enteropathy Associated with SLCO2A1 Gene（CEAS）Accompanied by Primary Hypertrophic Osteoarthropathy

要旨●患者は73歳，男性．12歳時貧血，貧血症状でCEAS発症．以後19年間，頻回の入院加療，鉄剤静注，輸血などを必要とした．29歳時胃潰瘍として胃切除（H. pylori抗体陰性）．30歳時，回腸─回腸吻合術施行，31歳時，BL（blind loop）切除．65歳時，繰り返すイレウス症状のため吻合部の経肛門的内視鏡的バルーン拡張術（EBD）を施行した．31歳時のBL切除後の42年間は鉄剤内服のみにて，貧血症状なく，中等度の労働に従事し，通常の社会生活を送っている．また，EBD後現在までの8年間は無症状で吻合部狭窄も解除した状態が持続している．以上，CEASの中では，良好な経過をたどっている1例の臨床経過と切除標本，X線造影検査・内視鏡検査の画像を提示し，考察を加えた．なお，合併した肥厚性皮膚骨膜症（PHO）の脳回転状頭皮の診断にヘリカルCTが極めて有用であった．

　A 73-year-old male patient developed CEAS（chronic enteropathy associated with SLCO2A1 gene）at age 12, and he had presented with anemia and anemic symptoms. Over the next 19 years, he was frequently hospitalized for treatment, and he received intravenous injections of iron preparations and blood transfusions. At age 29, he underwent gastrectomy because of a gastric ulcer（Helicobacter pylori antibodies：negative）. At ages 30 and 31, he underwent ileoileostomy（ileal-ileal anastomosis）and blind loop resection, respectively. At age 65, he underwent EBD（endoscopic balloon dilatation）at the anastomosis site because of repeated ileus symptoms. After 42 years of blind loop resection, he was able to be managed with oral administration of iron preparations. With no anemic symptoms, he was able to engage in moderate-level labor and lead a normal life. Over the 8 years since EBD and till date, he has been symptom-free, and no anastomotic stricture has recurred.

　Here, I report on the clinical progress of a patient with CEAS that followed a favorable course, together with a presentation of resected specimens, X-ray, and endoscopic images, and a discussion of the details. The results also indicate that helical computed tomography was extremely useful for the diagnosis of cutis verticis gyrata accompanying primary hypertrophic osteoarthropathy.