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目的探讨散发型内脏肌病的临床病理特点及其与继发性肌病的鉴别诊断。方法对1例散发型内脏肌病的尸体解剖标本通过光镜、电镜、组化及免疫组化等方法进行组织学观察。结果组织学特点为:消化管平滑肌细胞萎缩变性、纤维化,VG染色红染的胶原纤维呈蜂窝状排列,免疫组化Actin在肌层呈散在弱阳性表达。电镜下,胶原纤维分割包绕平滑肌细胞,肌膜不连续,肌丝紊乱,线粒体扩张,空泡变性。结论散发型内脏肌病为一种罕见疾病,根据其组织学特点,结合频繁呕吐、假性肠梗阻等临床表现,可以作出明确的病理诊断。
Objective To investigate the clinicopathological features of sporadic visceral myopathy and its differential diagnosis with secondary myopathy. Methods The autopsy specimens of one case of sporadic visceral myopathy were observed by light microscope, electron microscope, histochemistry and immunohistochemistry. Results The histological features were as follows: atrophic degeneration and fibrosis of the smooth muscle cells of the digestive tract were observed. Collagen fibers stained red by VG were arranged in a honeycomb pattern. Immunohistochemically, Actin was weakly positive in the muscle layer. Electron microscopy, collagen fibers wrapped around smooth muscle cells, muscle membrane is not continuous, myofilament disorders, mitochondrial expansion, vacuolar degeneration. Conclusion Disseminated visceral myopathy is a rare disease. According to its histological features, combined with the clinical manifestations of frequent vomiting and pseudo intestinal obstruction, a clear pathological diagnosis can be made.