Riemann J F, Schmidt H, Liendl I
Fortschr Med. 1981 Aug 6;99(29):1134-6.
Amyloidosis nearly always goes along with manifestations of the gastrointestinal tract. It can mimic a variety of different clinical syndromes. Pathogenetically amyloidosis leads to an organ insufficiency due to local infiltration of amyloid or to replacement of tissue with amyloid fibrils. Characteristic ultrastructure is composed of an irregular network of fibrils without any periodicity. The topographic position gives an explanation for the intestinal symptoms. Involvement of the intramural nerve system is responsible for motility disorders like diarrhoea or constipation. In case of perivasal infiltration hemorrhage and inflammatory changes can be found, as well as malabsorption syndromes, when subepithelial amyloid layers are present. Electron microscopy therefore may contribute to the pathogenetic understanding of morphological findings in regard to clinical symptoms.
淀粉样变性几乎总是伴有胃肠道表现。它可模拟多种不同的临床综合征。从发病机制上讲,淀粉样变性由于淀粉样蛋白的局部浸润或淀粉样原纤维对组织的替代而导致器官功能不全。其特征性超微结构由不规则的无周期性的原纤维网络组成。其在体内的位置可解释肠道症状。壁内神经系统受累可导致诸如腹泻或便秘等运动障碍。在血管周围浸润的情况下,可发现出血和炎症变化,当存在上皮下淀粉样蛋白层时,还会出现吸收不良综合征。因此,电子显微镜检查有助于从发病机制上理解与临床症状相关的形态学发现。