散发型内脏肌病——附1例尸检报告及文献复习

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。

Sporadic Visceral Myopathy-A Necropsy Report and Literature Review

Objective To study the clinical Pathological characteristics of sporadic visceral myopathy and its differential diag nosis from secondary myopathy. Methods A case of sporadic visceral myopathy, Proved by postmortem examination, was stud ied by light microscopy, electron histochemistry and immunohistochemistry. Results The histological characteris tics of the disease are atrophy, degeneration Of smooth muscle cells, fibrosis, honeycomd Pattem arrangement of collagen fibers with VG red staining, weak positive immunohistochemical reaction of actin in muscular layer; under electron microscope,smooth muscle cells surrounded by collagen, discontinuity of muscular memdrane, disorder of myofilament, dilation and vacuolar degener ation of mitochondria were seen. Thereby, the differential diagnosis from secondary myopathy could be Conclusions Sporadic visceral myopathy is a rare disease. Based on the histological characteristics and clinical manifestations such as frequent vomiting, pseudo intestinal obstruction, etc. a definite Pathological diagnosis of the disease can be made.