肝豆状核变性患儿肝脏病理及超微结构

PATHOLOGICAL AND ULTRASTRUCTURAL OBSERVATION OF THE LIVER OF A CHILD WITH WILSON'S DISEASE

目的对肝豆状核变性患儿的肝脏进行病理及超微结构观察,探讨肝脏病理在肝豆状核变性诊断和治疗中的价值。方法对1例亲体部分肝移植术后摘除的肝豆状核变性的肝脏组织进行HE、MASSON、TIMM、PAS染色等病理检查,并通过H-7500透射电镜进行肝细胞超微结构观察。结果光镜下肝细胞表现为不同程度的退行性变,胶原纤维增生以及典型假小叶形成;Timm's染色阳性,发现不均匀分布黑色颗粒或团块样物质沉积;PAS染色则普遍缺乏特异性染色;超微结构显示线粒体形态多样,体积增大,溶酶体增多,粗面内质网管腔扩张,附着核糖体的脱颗粒,以及胞质水肿,质膜溶解。肝脏病理及超微结构均提示患儿肝脏进入肝硬化阶段。结论肝脏病理检查对于肝豆状核变性诊断以及治疗方式的选择有重要价值。

Objective To observe the pathological changes of the liver of Wilson＇s disease in children,and evaluate the value of liver pathology in diagnosis and treatment of Wilson＇s disease. Methods The liver tissue of a child patient with Wilson＇s disease was taken after living related liver transplantation（LRLT）. The pathological changes of the liver were observed under the light microscope with HE, Masson, Timm＇s, and PAS staining, and transmission electron microscope （TEM）. Results Microscopically, different degrees of hepatocytic degenerative changes and hyperplasic fibrous tissue and typical false lobule formation could be seen. Timm＇s staining result showed uneven distributed black granular deposits in the hepatocytes. No specific PAS staining was observed. Utrastructurally, the mitochondria were increased in volume and dramatically different in shape. The number of lysomes were increased. Cisterna distension and degranulation were seen in endoplasmic reticulum. Cellular edema and cell membrane lysis were also found. Conclusion Pathological examination is of usage in the diagnosis and treatment of Wilson＇s disease.