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目的研究异常代谢产物沉积性肝病的肝组织超微病理及组织病理改变特征,结合临床资料探讨其诊断意义。方法2005-08—2006-03,对华中科技大学附属同济医院儿科8例临床拟诊为代谢性肝病患儿进行肝组织活检,分析肝组织病理及超微病理特征。结果普通病理报告提示糖原累积病2例,尼曼-匹克病1例,代谢性肝病1例,慢性轻型普通型肝炎1例,其余3例无特别提示。超微病理报告提示糖原累积病3例,脂质沉积性肝病5例。3例以肝细胞内糖原沉积为主要特征者诊断为糖原累积病,其中,1例肝细胞内大量PAS阳性物质,脂肪变性明显,无纤维组织增生,考虑为Ⅰ型;1例脂肪变性较轻而纤维化明显,考虑为Ⅲ型;另1例电镜发现肝细胞浆内单颗粒糖原明显增加,粗面和滑面内质网扩张,Disce间隔和部分肝细胞间纤维成分增多,部分细胞脂滴增加,考虑可能为Ⅱ型或其他轻型糖原累积病。5例以肝细胞浆内多个膜包裹的电子透亮空泡和泡内或疏松或致密高电子密度物质沉积为主要特征者诊断为脂质沉积性肝病,其中1例光镜见大量典型泡沫细胞而诊断为尼曼-匹克病,其余4例分类不明。结论电镜检查肝组织超微结构对于代谢性肝沉积病的诊断较光镜检查更有帮助,可明显提高诊断率。

Pathologic and ultromicro-pathologic feature s of liver tissues in children with metabolic depositing liver disease

??AbstractObjectiveTo study the features of pathologic and ultramicro-pathologic changes of abnormal metabolic products depositing liver disease,and investigate its diagnostic significance on the basis of the clinical data.MethodsFrom Aug 2005 to Mar 2006,8 children in our hospitoal who were diagnosed as metabolic liver disease according to their clinical manifestations were carried out liver biopsy.The features of pathologic and ultramicro-pathologic changes of liver were analyzed.ResultsOrdinary pathologic reports indicated 2 cases of glycogenic thesaurismosis,1 Niemann-Pick disease,1 metabolic liver disease;1 chronic mild ordinary hepatitis,and without special indication in other 3 cases.The reports of ultramicro-pathology showed 3 cases of glycogenic thesaurismosis and 5 cases of liver diseases of lipidoses.Three cases were featured as the glycogenic deposite in hepatocytes,so they were diagnosed as glycogenic thesaurismosis.Among them,1 was considered as type I because there were massive PAS positive material in hepatocytes with obvious adipose degeneration and without fibroplasia,1 was type ?? with apparent fibrosis and slight fatty degeneration,and another was considered as type ?? or other slight glycogenic thesaurismosis,whose appearance was evident increase of single granulometric glucogen in cytoplasm of hepatocytes and distension of rough and smooth endoplasmic reticulum with increase of lipid droplet in partial cells and increase of fibra element in Disce septa and between some hepatocytes.Five cases were diagnosed as liver diseases of lipidoses,whose key features were that there were several electronic transparent and bright vacuolus in cytoplasm of hepatocytes which were encapsuled by membrane and filled with porous or compact high density electronic substance.And among them,1 was Niemann-Pick disease because many typical foam cells ere observed under light microscope,and the classification of the other 4 cases was not clear.ConclusionExamining the ultramicro-structure of liver tissue by electronic microscope is more helpful to the diagnosis of liver metabolic thesaurismosis,and the rate of final diagnosis is improved greatly.