| BackgroundPrimary biliary cirrhosis(PBC) is an important type of autoimmune liver diseases.The disease is reported had a higher incidence in Western countries than it inChina.The annual incidence rate changes from0.39to1.5cases per millionpopulation in west countries,while there is no precise data of the annual incidencerate in China.Most of patients are middle-aged women, while one in ten of all aremale patients. However,because of increasing awareness of PBC on clinic and theadvance of medical technology, more PBC cases have been diagnosed in recentdecades.The main pathology of liver tissue of PBC is chronic non-suppurativeinflammation of intrahepatic bile duct.Its important markers are anti-mitochondrialantibodies (AMA) and the M2type (AMA-M2).When both of them are negative,wemust detect liver tissue by biopsy.The continuing actions of autoimmune antibodiesresults in progressive destruction of bile ducts, cirrhosis, portal hypertension andliver failure eventually.According to the U.S. Food and Drug Administration,Ursodeoxycholic (UDCA) is the only effective drug for PBC.If it is used at earlytime, the prognosis is good and the liver function can be maintained for a long time inthe compensatory phase.When treatment begins in icteric period, the prognosis is not good and liver transplant will be the only effective treatment of end-stage PBC.Soearly diagnosis and early treatment of PBC is important.In our study, the relationshipbetween clinical and pathologic characterisitic of PBC will be showed,to screenindicator for PBC.Reason treatment is the basic treatment,but he pathogenesis of PBC remainsunknown up to date.Histological evidence of liver biopsy supports that immunemechanisms involved in.So finding the reason is the most important thing.Lysosomalmembrane protein2(LAMP2) gene locates on Xq24-25, mainly expressed inplacenta, lung and liver.And it is one of the main components of the lysosomalmembrane.It is considered as a simple structure protein in past, but recent studieshave found that LAMP2also involved in the regulation of autoimmune diseases.It isa new subtype of anti-neutrophil cytoplasmic antibody (ANCA).As an autoimmunehepatic disease,may PBC have some relationship with LAMP2?In China,there arefew reports about relationship between the two.In our study, the feature of PBC willbe showed all-round from clinical and pathologic characterisitic to the effect ofabnormal LAMP2expression PBC.Our aim is to study the features of patients withPBC including clinical symptoms, lab tests and pathology,as well as to research thecause of PBC.ObjectionTo study the correlation among hepatic pathohistology of biopsies, expression ofLAMP2and lab tests in patients with PBC.MethodsChecked in45patients who were diagnosed as PBC with liver biopsy at the firstaffiliated hospital of Zhengzhou University from June2003to December2012.Registered their pathological staging, blood clotting, immune globulin andautoimmune antibody and other related indicators.And then observed expression ofLAMP2in liver tissue of patients with PBC by immunohistochemical methods. Atlast analyze the correlation among the three.Results1. As the severity of liver tissue of PBC patients, the expression of LAMP2in livershowed an increasing trend (χ2=20.534, P=0.002).The correlation coefficient (r) was0.555(P <0.05).2. γ-GT, ALP, DBIL and ALB were correlated with pathology staging of PBC.Thecorrelation coefficients were0.318,0.305,0.410and-0.295respectively (P valueswere0.033,0.041,0.005, and0.049respectively).The median of DBIL wasstatistically significant different in different pathological stagings(χ2=8.691,P=0.013).And the area of ROC of γ-GT to Ⅲ+Ⅲ staging is greater than0.7.PT, FIB,IgG, IgM, titers of ANA, AMA, AMA-M2all were not associated with liverpathology staging.3. γ-GT, ALP, DBIL and ALB were correlated with LAMP2expression.Thecorrelation coefficients were0.578,0.522,0.479and-0.335respectively(P=0.000,0.000,0.001and0.024respectively).The median of γ-GT, ALP, DBIL and ALBwere different at different level of LAMP2expression(P=0.001,0.001,0.006and0.021respectively).PT, FIB, IgG, IgM, titers of ANA, AMA, AMA-M2all were notassociated with the expression of LAMP2.Conclusions1. The expression of LAMP2in liver tissue has relationship with live function andprogression of PBC, so it is assumed that the abnormal expression of LAMP2involve in the development of PBC.2. γ-GT, ALP, DBIL and ALB have relationship with the progression of PBC;and thearea of ROC of γ-GT to Ⅲ+Ⅲ staging is greater than0.7;Solive function is helpfulto judge hepatic pathology,especially γ-GT. |
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