膽道閉鎖同學(xué)版

1、Biliary Atresia 李英存重慶醫(yī)科大學(xué)兒科學(xué)院外科教研室Anatomynormal biliary tractbiliary atresiaBabies with biliary atresia目 的掌握膽道閉鎖的病理、臨床表現(xiàn)及診斷要點(diǎn)熟悉膽道閉鎖的治療原則了解膽道閉鎖的病因及鑒別診斷IntroductionEtiologyPathologyClinical manifestationDiagnosisDifferential diagnosisTreatmentComplicationsIntroductionEtiologyPathologyClinical manifest

2、ationDiagnosisDifferential diagnosisTreatmentComplicationsDefinition Complete occlusion of partial or entire biliary tract, happened within 3 mos after birth.biliary atresiaIncidence ：1：8000-1：15000 Asia Europe & AmericaAge： soon after birthGender：male：female=1：1.56IntroductionEtiologyPathologyClini

3、cal manifestationDiagnosisDifferential diagnosisTreatmentComplicationsNot clear Viral infection: CMV(巨細(xì)胞病毒），RV(輪狀病毒） CMV: pros：Infection rate of CMV is higher in biliary atresia . cons：Animal model cant be established. RV (輪狀病毒） : Evidences：1）RV antibody was found in biliary atresia.2）RV RNA and gra

4、nules were detected in liver tissue of biliary atresia.3）Animal model has been established.4）Seasonality.Immune/autoimmmune injury1）Infiltration of monocytes （CD4+，CD8+）in porta hepatis(肝門）and portal area(匯管區(qū)）.2）Immature of NK cell.3) IFNgene expression increased in liver tissue of biliary atresia.4

5、）IFN-/- animal test.Ductal plate malformation: Mutation of Kartagener gene，Inv gene，HOX gene，CFC1 gene，JAG1 gene，et al.IschemiaRegarded as autoimmune disease in recent years Pathogenic factors（virus、toxin）Antigen changes on epithelium of biliary tract Antigen shedding（遞呈）T cell receptorTH1 cytokines

6、、TcInjury of epithelium and occlusion of biliary tractIntroductionEtiologyPathologyClinical manifestationDiagnosisDifferential diagnosisTreatmentComplicationspathologyGrossAtresia of intrahepatic biliary tract. Fibrosis of gallbladder, white bile. Hepatomegaly with tubercles.HistologyChronic inflamm

7、ation of porta hepatis, triangular cord with irregular bile duct.Portal area inflammation with fibrosis and ductular proliferation .Liver cirrhosis of biliary atresiaNormal liver tissueNormal hepatic lobule Fibrosis and ductular proliferation in portal area of biliary atresiaprogressive inflamation

8、of biliary tract and liver fibrosisInflamation of biliary tractLiver cirrhosis CholestasisInjury of biliary tract and hepatocyteObstruction of biliary tractClassificationType : Patent proximal ducts with distal fibrosis (膽總管閉鎖,5-10%）(可矯治型）ClassificationType : Obliteration of proximal ducts with pate

9、nt distal system（肝管閉鎖，2-3%） 不可矯治型ClassificationType : Complete obliteration of biliary ducts （肝門部閉鎖，85-90%） 不可矯治型IntroductionEtiologyPathologyClinical manifestationDiagnosisDifferential diagnosisTreatmentComplicationsClinical manifestation（symptoms) persistent jaundice:Happened soon after birth,pers

10、istent. Clay-coloured faeces(陶土樣大便）: Companied with jaundice,sometimes light yellow in the late stage of disease.Symptoms often occur soon after birth嬰兒糞便比色卡Clinical manifestation（symptoms) persistent jaundice:Happened soon after birth,persistent. Clay-coloured faeces(陶土樣大便）: Companied with jaundice

11、,sometimes light yellow in the late stage of disease. Dark urine:Like strong tea.Symptoms often occur soon after birthClinical manifestation (signs) jaundice：persistent. Hepatomegaly（肝大）: Splenomegaly(脾大）and ascite may happen at the late stage of disease.IntroductionEtiologyPathologyClinical manifes

12、tationDiagnosisDifferential diagnosisTreatmentComplicationsBlood test：liver function（TB 、 DB、ALT、AST、 GGT、 ALP elevation）,DB/TB50% is important for diagnosis.Clinical manifestation + Lab testColour Doppler Ultrasound(彩超）：First choice 1.The gallbladder is small size or not observed ,and doesnt contra

13、ct well; 2.Lumen of common bile duct does not exit, or the wall es thickened; 3.The echo of porta hepatis triangular is enhanced .Gallbladder of Fasting and postprandial Cholangiogram（膽道造影）: Gold standard The cholangiogram is done using a small catheter introduced into the fundus of the gallbladder.

14、Normal biliary ductProximal biliary duct obliterationComplete obliteration of biliary ductsRadioisotope excretion studies（放射性核素顯像）： Not specificDuodenal fluid aspiration(十二指腸引流液分析)： Hard to useMagnetic resonance cholangiopancreatography （MRCP）：Liver biopsy：IntroductionEtiologyPathologyClinical manif

15、estationDiagnosisDifferential diagnosisTreatmentComplicationsNeonatal hepatitis:Similarity : Jaundice happened soon after birth.Discrimination : (more likely to be BA) (1) persistent jaundice. (2)Clay-coloured faeces began early. (3)Hepatomegaly（4cm) with solid and clear edge. (4) Lab test DB elevat

16、ed persistently ALP40U(金氏） GGT 300U/LCholangiogram is gold standard for differential diagnosisCongenital biliary dilatationSimilarity : Jaundice may happened soon after birth.Discrimination: (more likely to be BA)persistent jaundice.Clay-coloured faeces.Open biopsy and Cholangiogram is gold standard

17、 for differential diagnosis3. TPN related cholestasis(1)History:TPN treatment(2 weeks).(2)Clinical manifestation (TPN related cholestasis) 1)Jaundice(If changeable). 2)Hepatomegaly is not very obvious, with soft edge4. Biliary tract is pressed by tumor or lymph nodeIntroductionEtiologyPathologyClini

18、cal manifestationDiagnosisDifferential diagnosisTreatmentComplicationsPrinciples：Early diagnosis and early operation.Prognosis:Survival rate 20% in the first year without operationOperation age:90 days, 60 days is preferOperation methods 1.Hepatico-enterostomy(肝管空腸吻合術(shù)）for correctable-type. (less common) 2.Hepatic porto-enterostomy(肝門空腸吻合術(shù)，Kasai術(shù)）for non-correctable types. (common).5-year survival rate