CAJ | 학술논문

目的探讨线粒体细胞病肌肉组织超微结构特征及其诊断价值.方法对33例线粒体细胞病的肌肉标本进行了系统的常规病理学和电镜超微结构观察.结果主要病理学改变为:改良Gomori染色可见破碎红边纤维,SDH染色可见深染肌纤维,COX/SDH双重染色可见COX浅染而SDH深染肌纤维.电镜观察可见肌膜下、肌原纤维之间线粒体数量增多,线粒体结构异常,晶格状包涵体,线粒体嵴呈同心圆样板层排列,肌膜下糖脂复合物数量增多.结论发现数量增多而且结构异常的线粒体、电子致密的线粒体颗粒和晶格样包涵体,可提供支持诊断的证据.
목적 탐토선립체세포병기육조직초미결구특정급기진단개치.방법 대33례선립체세포병적기육표본진행료계통적상규병이학화전경초미결구관찰.결과 주요병이학개변위:개량Gomori염색가견파쇄홍변섬유,SDH염색가견심염기섬유,COX/SDH쌍중염색가견COX천염이SDH심염기섬유.전경관찰가견기막하、기원섬유지간선립체수량증다,선립체결구이상,정격상포함체,선립체척정동심원양판층배렬,기막하당지복합물수량증다.결론 발현수량증다이차결구이상적선립체、전자치밀적선립체과립화정격양포함체,가제공지지진단적증거.
Objective To investigate the ultrsstructural features of mitochondrial cytopathies and its diagnostic value.Methods Muscle biopsy specimens from 33 cases of mitochondrial cytopathies were examined by routine pathological and electron microscopic examinatiom.Results The main pathologic changes included ragged red fibers in modified Gomori staining,hyper-intense staining myotibers in SDH,COX-negative fibers while dark counterstaining with SDH in COX/SDH double staining technique.Ultrastructural findings included subsarcolemmal and intramyofibrillar prolireration of mitochondrin and the appearance of abnormal mitochondria,paracrystalline inclusions,concentric dystrophic cristae and excessive subsarcolemmal glycolipid compounds in subsarcolemmal.Conclusion The presences of proliferation and abnormality of mitochondria,electro-dense granule and paracrystalline inclusions in mitochondria provide key diagnostic evidence for the diagnosis ofthis ditease.