Wilson氏病多器官的病理和超微病理学研究  

Clinical, pathological and ultramicropa-thological studies on hepatolenticular degeneration.

作　　者：张柏蘋 梁秀龄[1] 刘焯霖[1] 刘海荣[1] 潘锡榜 

机构地区：[1]中山医科大学神经病学教研室

出　　处：《中国神经精神疾病杂志》1992年第1期18-20,共3页Chinese Journal of Nervous and Mental Diseases

摘　　要：本文对1例HLD死者进行了病理和电镜研究。肉眼可见结节性肝硬化,脾肿大,脑萎缩和豆状核软化灶等。光镜显示坏死后性肝硬化,可见铜离子在肝细胞及其周围沉积;在基底节区可见散在的神经细胞变性,继发性的神经胶质增生,并可找到Alzheimer Ⅱ型细胞。对5种器官(肝、肾、脑、脾和皮肤)进行电镜观察,在肝、肾、皮肤组织均可见散在的高电子密度物质沉积。本研究是国内首次对HLD进行多器官的超微病理学观察,它对促进本病的研究具有重要意义。In this thesis the pathological and electron-microscopical studies on a dead patient with hapatolenticular degeneration (HLD) were carried out.To the naked eye nodular cirrhosis,splenomegaly,brain atrophy,and malacia of lenticular nuclei etc were found in autopsy.The light microscopy showed the postnecrotic cirrhosis,in which copper granules deposited in the outskirts cells of lobuli hapatic.Scattered degneration and necrosis of neuros,reactive astrocytic gliosis and nodular gliosis were within the basal nuclei,and there were Alzheimer II type cells.Electronmicroscopic examination of 5 organs (Liver,kidney,brain,spleen,and skin) was performed.Scattered high electronic density granules were found in liver,kidney,and skin tissues.This study was the first investigation of the ultra-microstucture in multiple organs of HLD in China and has an important significance for the advance research of this disorder.

关 键 词：病理学 超微病理学 肝豆状核变性 

分 类 号：R575.240.2[医药卫生—消化系统]