Hitzman J L, Weiland L H, Oftedahl G L, Lie J T
Mayo Clin Proc. 1979 Apr;54(4):251-7.
A middle-aged patient with "brown bowel syndrome" or gastrointestinal ceroidosis manifested as malabsorption of undetermined cause is described. Autopsy revealed involvement of the entire gastrointestinal tract and unusual cardiac findings. Microscopically, the pigment responsible for the discoloration is a lipofuchsin that is deposited in the smooth muscle cells of the gastrointestinal tract. In all reported cases, ceroidosis was associated with some abnormality of the gastrointestinal tract--that is, malabsorption, steatorrhea, or gross local disease--with the possible exception of prolonged malnutrition. This case is also unusual because of the unexplained multifocal proliferative endocarditis. Vitamin E deficiency may be the common denominator of all these various disorders.
本文描述了一名中年患者,患有“棕色肠综合征”或胃肠道蜡样质沉积症,表现为不明原因的吸收不良。尸检显示整个胃肠道均受累,并伴有不寻常的心脏表现。显微镜下,导致变色的色素是一种脂褐素,沉积在胃肠道的平滑肌细胞中。在所有已报道的病例中,蜡样质沉积症都与胃肠道的某些异常有关——即吸收不良、脂肪泻或严重的局部疾病——长期营养不良可能是个例外。该病例也很不寻常,因为存在无法解释的多灶性增殖性心内膜炎。维生素E缺乏可能是所有这些不同疾病的共同特征。