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糖原储积病Ⅰ型,为遗传性代谢障碍疾病。本文着重报告本病肝细胞超微结构改变特点。材料为2例患儿肝活检标本。经戊二醛、O_SO_4双固定,按电镜样品制备常规脱水、色埋。I_μ薄切片经甲苯胺兰—Pyronin染色,光镜观察定位。超薄切片用铅、铀双染,国产DXB_2-12型电镜观察。光镜下肝细胞明显肿胀,其大小可为正常肝细胞体积2-3倍,排列如植物细胞样(图I)。部分深染肝细胞胞浆内充满致密物,多数肝细胞内脂滴异常增多,其余肝细胞表现程度不同混肿和颗粒性变。电镜下,光镜所见深染细胞为胞质内糖原颗粒大量堆积,其间伴有糖原自噬泡及小泡状滑面内质网(图2),有的肝细胞胞质内大片糖原高度聚集,形成糖原板块(图3),多数肝细胞胞质内脂滴显著增多,脂滴周围常伴随
Glycogen storage disease type Ⅰ, a genetic metabolic disorder. This article focuses on the report of liver disease ultrastructural changes. Materials for the two cases of liver biopsy specimens. The glutaraldehyde, O_SO_4 double fixed, electron microscopy sample preparation conventional dehydration, color buried. I_μ thin sections by toluidine blue-Pyronin staining, light microscopy positioning. Ultrathin sections with lead, uranium double staining, domestic DXB_2-12 type electron microscopy. Light microscopic liver cells were significantly swollen, the size of normal liver cells 2-3 times the size, arranged as a plant cell-like (Figure I). Some deep-stained hepatocytes were filled with dense cytoplasm, most of the abnormal increase in lipid droplets within the liver cells, and the remaining hepatocytes showed varying degrees of mixed swollen and granular changes. Under electron microscope, the deep-seeded cells seen by light microscopy were a large number of accumulation of glycogen granules in the cytoplasm, accompanied by autophagic vesicles and vesicular smooth endoplasmic reticulum (Figure 2), some large cytoplasm of hepatocytes Glycogen highly aggregated to form glycogen plate (Figure 3), the majority of hepatocytes in the cytoplasm lipid droplets increased significantly, often accompanied by lipid droplets around