Liver Pathology And Ultrastructure Of Wilson's Disease In Children

Objectives: To observe the pathology and ultastructure of wilson's disease in children ,and survey the relationship between the liver pathology and clinical grade of liver function of Wilson's disease for the pathological diagnosis of Wilson' disease.Method: The Sample of this study came from the Living related liver transplantation (LRLT) of the patient and her mother. As the anatomic position of the wilson's disease patient's liver, it divided into 6 groups, which are upper portal vein area, below portal vein area, left upper area, left below area, right upper area, right below area; to compared with donator's liver. Observed under the light microscope which are HE, Masson, Timm, and PAS stained , and under the scan electron microscope (SEM) and transmission electron microscope (TEM) .Results:1. The most specimen of patient's liver show decreased physical volume, rigid quality as the forehead, widespread of yellow nodules distribution surrounding the fibers.2. According to HE and Masson staining , the pathologic change of liver is diversify, such as Liver cells shape changed, showed vacuole, amalgamation, nucleus disintegrated or disappeared and false hepatic lobule which is deficiency of center vein and the shape and size of hepatocyte in the false lobule is different. It also shows the fiber hyperplasic which is encircled the false lobule, at Portal triad area found that artery, vein and bile duct arranged disorderly and their endothelium damnification. There is typical difference in shape of hepatocyte between the receptor and the donator. 3. Timm's stainning, the hepatocyte of receptor shows typical black deposit of granules and conglomerations that is irregular around the portal vein and hepetocytes in the false false lobule; the hepatocyte of donator stained weak, and no typical black deposit of granules and conglomerations. 4. Pas stainning: Hepatocyte of receptor is deficiency of specific stain, only the false lobule of hepetocytes is pink and irregular stained, and show the fuction of hepotacyte at false lobule increased, but that of donator is pink uniformly. 5. Sem also shows that the liver cells shape changed ,false hepatic lobule existed, and fiber hyperplasic. 6. Ultrastructure :The mitochondria shows various profile such as the increases volume, electron opacity of the matrix and the intercristal spaces appeared dilated, the inner and outer membranes separated, misshapen, giant elongated, ballooned ,or bizarre mitochondria. The mitochondrial inclusions is the type of paraerystallone and rod-like. Lysosome increased and was larger and full of rough granules. Otherwise, Trans-Golgi complexs show the shape of flat vesicle variance and content residual. endoplasmic reticulum cisterna distended and the ribosome brushed off . 7. Characteristic of copper depoist and damnification of WD: Timm's stainning shows that the copper deposit is asymmetric which is related with the damnification. The pathology is distinct at different part. The shape of hepatocyte and fiber hyperplasia is more seriouls at portal vein part which is confirmed by HE,Masson stainning ,SEM and TEM .Conclusions1. Most WD in children have a liver symptom firstly. The early pathology is nonspecific, the late pathology show liver fibrosis and cirrhosis in some extent . 2. The atom Cu distributed at the liver of WD in children is asymmetric which is excessive at portal vein, nodus, and fiber hyperplasia part. In hepatocyte Cu assembled at lysosome gradually with the course of diseases.3. There may be difference between the clinical grade and the pathology grade among WD in children. 4. Mitochondia and lysosome were the most serious organelles in the hurts of hepatocyte ultrastructure. The changes of Golgi maybe play an important role in mechanism.