Lin C N, Huang A H, Hsu S I, Lee C, Liang A I, Kuo C I, Chuang S S
Department of Pathology, Chi-Mei Foundation Hospital, Tainan, Taiwan R.O.C.
J Formos Med Assoc. 1993 Dec;92(12):1090-4.
Brown bowel syndrome is a rare intestinal disorder associated with the deposition of lipofuscin pigment in the smooth muscle cells. We report two such cases presenting with intestinal pseudo-obstruction, abdominal pain, and body weight loss. Both cases had malabsorption and fatty liver. Exploratory laparotomy revealed brownish discoloration of the small bowel wall and enlargement of mesenteric lymph nodes. Light microscopy, autofluorescence and ultrastructure studies confirmed the deposition of lipofuscin pigments in the intestinal muscle cells and reticuloendothelial cells of mesenteric lymph nodes. In addition, the calf muscle biopsy of case 1 displayed myopathy and fatty replacement. Skeletal muscle strength of both patients was partially restored after parenteral and oral vitamin E supplement and other conservative treatment, but gastrointestinal symptoms of both patients continued to deteriorate. Thus, brown bowel syndrome associated with prolonged and severe malnutrition and possibly vitamin E deficiency appears only partially responsive to vitamin E supplementation.
棕色肠综合征是一种罕见的肠道疾病，与脂褐素色素在平滑肌细胞中的沉积有关。我们报告了两例表现为肠道假性梗阻、腹痛和体重减轻的此类病例。两例均有吸收不良和脂肪肝。剖腹探查发现小肠壁呈褐色变色，肠系膜淋巴结肿大。光学显微镜、自发荧光和超微结构研究证实脂褐素色素沉积在肠肌细胞和肠系膜淋巴结的网状内皮细胞中。此外，病例1的小腿肌肉活检显示有肌病和脂肪替代。经肠外和口服维生素E补充剂及其他保守治疗后，两名患者的骨骼肌力量部分恢复，但两名患者的胃肠道症状仍持续恶化。因此，与长期严重营养不良以及可能的维生素E缺乏相关的棕色肠综合征似乎仅对维生素E补充剂有部分反应。
