Ruchti C, Eisele S, Kaufmann M
Institute of Pathology, University of Bern, Switzerland.
Arch Pathol Lab Med. 1990 Jan;114(1):76-80.
A 52-year-old man, suffering for years from malabsorption due to endemic sprue, developed progressive bowel dysfunction, ie, recurrent ileus and intestinal pseudo-obstruction. Because of partial volvulus formation, ileocecal resection was performed. Histopathologic examination of the resected specimen revealed signs of advanced brown bowel syndrome, with excessive deposits of ceroid-lipofuscin in, and a considerable loss of, smooth-muscle cells and myofibrils. The patient died after surgery, and at autopsy a systemic ceroid lipofuscinosis of smooth-muscle cells was detected. Neuronal ceroid lipofuscinosis was not observed. Serious and eventually fatal bowel dysfunction is rarely seen in brown bowel syndrome, but may occur in advanced stages. Since treatment with vitamin E appears to exert a favorable effect, early diagnosis of brown bowel syndrome seems to be very important.
一名52岁男性，因地方性糙皮病多年来一直患有吸收不良，出现了进行性肠道功能障碍，即反复性肠梗阻和假性肠梗阻。由于形成了部分肠扭转，进行了回盲部切除术。切除标本的组织病理学检查显示有晚期棕色肠道综合征的迹象，在平滑肌细胞和肌原纤维中有过多的脂褐素沉积，且平滑肌细胞和肌原纤维大量丧失。患者术后死亡，尸检时发现平滑肌细胞全身性脂褐素沉着症。未观察到神经元性脂褐质沉积症。严重且最终致命的肠道功能障碍在棕色肠道综合征中很少见，但可能发生在晚期。由于维生素E治疗似乎有良好效果，棕色肠道综合征的早期诊断似乎非常重要。
