|
|
Pathologic and ultromicro-pathologic features of liver tissues in children with metabolic depositing liver disease.
ZHOU Hua,FANG Feng,XU San-qing
2007, 22(05):
370-372 .
AbstractObjectiveTo study the features of pathologic and ultramicro-pathologic changes of abnormal metabolic products depositing liver disease,and investigate its diagnostic significance on the basis of the clinical data.
MethodsFrom Aug 2005 to Mar 2006,8 children in our hospitoal who were diagnosed as metabolic liver disease according to their clinical manifestations were carried out liver biopsy.The features of pathologic and ultramicro-pathologic changes of liver were analyzed.
ResultsOrdinary pathologic reports indicated 2 cases of glycogenic thesaurismosis,1 Niemann-Pick disease,1 metabolic liver disease;1 chronic mild ordinary hepatitis,and without special indication in other 3 cases.The reports of ultramicro-pathology showed 3 cases of glycogenic thesaurismosis and 5 cases of liver diseases of lipidoses.Three cases were featured as the glycogenic deposite in hepatocytes,so they were diagnosed as glycogenic thesaurismosis.Among them,1 was considered as type I because there were massive PAS positive material in hepatocytes with obvious adipose degeneration and without fibroplasia,1 was type Ⅲ with apparent fibrosis and slight fatty degeneration,and another was considered as type Ⅱ or other slight glycogenic thesaurismosis,whose appearance was evident increase of single granulometric glucogen in cytoplasm of hepatocytes and distension of rough and smooth endoplasmic reticulum with increase of lipid droplet in partial cells and increase of fibra element in Disce septa and between some hepatocytes.Five cases were diagnosed as liver diseases of lipidoses,whose key features were that there were several electronic transparent and bright vacuolus in cytoplasm of hepatocytes which were encapsuled by membrane and filled with porous or compact high density electronic substance.And among them,1 was Niemann-Pick disease because many typical foam cells ere observed under light microscope,and the classification of the other 4 cases was not clear.
ConclusionExamining the ultramicro-structure of liver tissue by electronic microscope is more helpful to the diagnosis of liver metabolic thesaurismosis,and the rate of final diagnosis is improved greatly.
|