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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。
Objective To study the ultrastructural and histopathological changes of liver tissue in patients with ancillary liver diseases with abnormal metabolites, and discuss the significance of its diagnosis with clinical data. Methods From August 2005 to June 2006, 8 biopsy specimens of children with metabolic liver disease were collected from pediatric department of Tongji Hospital Affiliated to Huazhong University of Science and Technology. The pathological and ultrastructural features of liver were analyzed. Results The common pathological reports suggested glycogen storage disease in 2 cases, Niemann-Pick disease in 1 case, metabolic liver disease in 1 case, and chronic mild hepatitis in 1 case. The remaining 3 cases were not prompted. Superficial pathology report suggestive of glycogen storage disease in 3 cases, 5 cases of lipid deposition of liver disease. 3 cases of glycogen deposition in hepatocytes as the main feature of diagnosis of glycogen storage disease, of which a large number of PAS-positive cells in liver cells, fatty degeneration was obvious, no fibrous tissue hyperplasia, considered as type Ⅰ; 1 case of steatosis Lighter and obvious fibrosis, considered as type III; the other electron microscopy showed a single hepatocyte plasma glycogen significantly increased, rough and smooth endoplasmic reticulum dilatation, interstitial Disce and part of the increase in fiber components between the liver cells, part of Cell lipid droplets increased, consider may be type Ⅱ or other light glycogenosis. Five patients were diagnosed as lipid-induced hepatopathy with electronic translucent vacuoles and deposits of bubbles or loose or dense high electron density material wrapped by multiple membranes in liver cytoplasm. Among them, a large number of typical foam cells The diagnosis of Niemann-Pick disease, the remaining 4 cases of unknown classification. Conclusion Electron microscopic examination of the ultrastructure of liver tissue is more helpful than light microscopy in the diagnosis of metabolic hepatosplenosis and can significantly improve the diagnostic rate.