糖原累积病Ⅰ型肝脏组织的超微结构观察

Ultrastructural study on live tissue in a case of glycogen storage disease typeⅠ

用透射电镜观察一例经治疗后糖原累积病Ⅰ型(GSD-Ⅰ)患儿肝组织的超微结构,发现肝细胞内有大量α-糖原颗粒聚集,并可见缺乏糖原的糖酵解区及脂滴。肝星形细胞(HSC)中有大量脂滴沉积。此结果说明,GSD-Ⅰ型患者可通过糖酵解取得部分能量,故经治疗后患者的肝功能可基本恢复正常。

The ultrastructure of the live tissue of a child with glycogen storage disease type Ⅰ （GSD- Ⅰ） after treatment was studied by electron microscopy. In the cytoplasm of hepatocytes, plenty of α- glycogen accumulated. Some glycogen-free areas and lipid droplets were encountered within the glycogen masses. Heaps of lipid droplets occurred in the hepatic stellate cells. This findings indicate that energy demand of hepatocytes in the case of GSD-Ⅰ can be partially compensated via glycolysis and after treatment the live functions of this child have approximately retum to normal.