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本文对14例wilson氏病肝脏进行了光镜和电镜观察,重点观察肝细胞、贮脂细胞(FSC)及窦周隙的超微结构改变,并探讨其发生机理。wilson氏病是一种常染色体隐性遗传性铜代谢障碍引起的疾病。铜主要沉积在肝、脑、肾。光镜下见肝细胞内散布着棕褐色颗粒,经红氨酸铜染色呈墨绿色。电镜下肝细胞内有大而高密度的溶酶体(图1)。yasuaki等对本病肝细胞内溶酶体部位进行微量元素测定,证实有多量铜沉积,这种含铜颗粒可以通过红氨酸染色在光镜下显示出来。所以血清铜降低、肝内铜沉积增多是wilson氏病所特有的。铜主要沉积在溶酶体内,铜不但使溶酶体功能受害,还能增加溶酶体膜的脆性致溶酶体破裂,落酶体内酶溢出导致细胞自溶死亡,提示肝细胞的损伤与铜的沉积有密切关系。
In this paper, 14 cases of Wilson’s disease liver were observed by light and electron microscopy, focusing on the liver cells, fat storage cells (FSC) and sinus ultrastructure of the ultrastructural changes, and explore its mechanism. Wilson’s disease is an autosomal recessive disease caused by copper metabolism disorders. Copper is mainly deposited in the liver, brain and kidney. Light microscope, see the liver cells scattered with brown particles, copper staining by erythorine was dark green. Electron microscope, there are large, high-density lysosomes in hepatocytes (Figure 1). yasuaki and other elements of the intrahepatic lysosome lysosome determination of trace elements, confirmed that there is a large amount of copper deposition, this copper-containing particles can be displayed by light microscope under erythrosine staining. Therefore, serum copper decreased intrahepatic copper deposition is increased wilson’s disease unique. Copper is mainly deposited in the lysosome, copper not only damage the lysosomal function, but also increase the fragility of the lysosomal membrane lysosomal rupture, enzyme excretion of enzymes in the body lead to autolysis death, suggesting that the damage of liver cells and copper The deposition is closely related.