Saito Yasushi, Hiramatsu Katsushi, Nosaka Takuto, Ozaki Yoshihiko, Takahashi Kazuto, Naito Tatsushi, Ofuji Kazuya, Matsuda Hidetaka, Ohtani Masahiro, Nemoto Tomoyuki, Imamura Yoshiaki, Nakamoto Yasunari
Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, 23-3 Matsuoka Shimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193, Japan.
Department of Pathology, University of Fukui Hospital, Fukui, Japan.
Clin J Gastroenterol. 2017 Aug;10(4):351-356. doi: 10.1007/s12328-017-0755-y. Epub 2017 Jun 29.
A 75-year-old man presented with abdominal distension, hypoproteinemia, ascites and a 35-mm mass in the small bowel mesentery. Laparotomy was performed, and he was diagnosed with sclerosing mesenteritis. His clinical condition improved, with computed tomography (CT) showing tumor shrinkage and decreasing ascites after administration of prednisolone; however, on drug withdrawal, abdominal fullness recurred and CT revealed an enlarging tumor and increasing ascites. Capsule endoscopy (CE) and double-balloon enteroscopy (DBE) were performed to further investigate hypoalbuminemia, which revealed white villi, white nodules, white debris, and mucosal edema in the jejunum. Biopsies from the jejunal mucosa demonstrated infiltration by chronic inflammatory cells consisting mostly of lymphocytes and plasma cells, with marked lymphangiectasia of the lamina propria and submucosa. A fecal alpha-1-antitrypsin clearance test revealed abnormal leakage from the gastrointestinal tract, confirming that hypoalbuminemia was secondary to protein-losing enteropathy (PLE). The incidence of sclerosing mesenteritis accompanied by PLE is very rare. Only six cases have been reported so far. CE and DBE were helpful for diagnosing this condition, and should be performed in patients in whom the cause of hypoalbuminemia is unknown, and in those with PLE.
一名75岁男性出现腹胀、低蛋白血症、腹水，小肠系膜有一个35毫米的肿块。进行了剖腹手术，他被诊断为硬化性肠系膜炎症。他的临床状况有所改善，计算机断层扫描（CT）显示在给予泼尼松龙后肿瘤缩小且腹水减少；然而，停药后，腹部胀满复发，CT显示肿瘤增大且腹水增多。进行了胶囊内镜检查（CE）和双气囊小肠镜检查（DBE）以进一步研究低白蛋白血症，结果显示空肠有白色绒毛、白色结节、白色碎片和黏膜水肿。空肠黏膜活检显示主要由淋巴细胞和浆细胞组成的慢性炎症细胞浸润，固有层和黏膜下层有明显的淋巴管扩张。粪便α-1-抗胰蛋白酶清除试验显示胃肠道有异常渗漏，证实低白蛋白血症继发于蛋白丢失性肠病（PLE）。伴有PLE的硬化性肠系膜炎症的发病率非常低。迄今为止仅报告了6例。CE和DBE有助于诊断这种情况，对于低白蛋白血症病因不明的患者以及患有PLE的患者应进行这两项检查。
