Nogami K, Kusachi S, Nunoyama H, Kondo J, Endo C, Yamamoto K, Murakami T, Tsuji T
First Department of Internal Medicine, Okayama University Medical School, Japan.
Jpn Heart J. 1996 Jul;37(4):483-94. doi: 10.1536/ihj.37.483.
Fibrotic change is one of the characteristic features of the heart with idiopathic dilated cardiomyopathy (DCM), but the extracellular matrix components have not been fully clarified. Fibronectin, laminin, and types IV, I and III collagen staining using the avidin-biotin-peroxidase complex method was conducted to study the localization of extracellular matrix components in biopsy specimens obtained from 7 patients with DCM. Fibronectin was observed in the endomysium and perimysium, co-existent with types I and III collagen; it was also distributed nonhomogeneously in the replacement fibrotic lesions. The staining intensity in a section varied substantially from location to location. Laminin surrounded the myocytes and was distributed in the endomysium localized together with type IV collagen, but it was not found in the perimysium. Weak but positive staining was observed in replacement fibrotic lesions in 2 patients. Similarly to laminin, type IV collagen staining was observed around the myocytes and in the endomysium. It was also observed in replacement fibrotic lesions in 4 patients either with or without laminin. Type I collagen, localized together with type III collagen, was distributed in both the endomysium and perimysium. It was also distributed nonhomogeneously in the replacement fibrotic lesions. Similar staining to type I collagen was seen for type III collagen. In summary, all examined components were observed in the fibrotic lesions with no primary deficit of any examined component being demonstrated. The variability of staining intensity and positive fibronectin staining suggests that the phases of fibrotic changes differed substantially from location to location.
纤维化改变是特发性扩张型心肌病（DCM）心脏的特征性表现之一，但细胞外基质成分尚未完全明确。采用抗生物素蛋白-生物素-过氧化物酶复合物法对7例DCM患者活检标本进行纤连蛋白、层粘连蛋白以及IV型、I型和III型胶原染色，以研究细胞外基质成分的定位。在肌内膜和肌束膜中观察到纤连蛋白，与I型和III型胶原共存；它在替代性纤维化病变中也呈非均匀分布。切片中的染色强度在不同位置差异很大。层粘连蛋白围绕心肌细胞，分布于与IV型胶原一起定位的肌内膜中，但在肌束膜中未发现。在2例患者的替代性纤维化病变中观察到弱阳性染色。与层粘连蛋白类似，IV型胶原染色在心肌细胞周围和肌内膜中可见。在4例有或没有层粘连蛋白的患者的替代性纤维化病变中也观察到IV型胶原染色。I型胶原与III型胶原一起定位，分布于肌内膜和肌束膜中。它在替代性纤维化病变中也呈非均匀分布。III型胶原的染色与I型胶原相似。总之，在纤维化病变中观察到所有检测成分，未发现任何检测成分存在原发性缺陷。染色强度的变异性和纤连蛋白阳性染色表明，纤维化改变的阶段在不同位置差异很大。
