The Clinicopathological Study Of Infantile Cholestasis

Object To evaluate the clinical features, etiology and liver pathological changes of infantile cholestasis and to demonstrate the value of a scoring system in the interpretation of histology to the diagnosis of infantile cholestasis. Methods Retrospectively analyzed the clinical and liver pathological information of 67 cases of infantile cholestasis in the children hospital of Zhejiang University from 2005 to 2009. Requirement:age1.0mg/dl or TB>5 mg/dl, DB/TB> 20%; unknown etiology or inefficiency after clinical treatment; have the pathological results by needle biopsy of liver; except choledochal cyst and hepatitis A,B,C,D,E virus. Result 1. Of 54 cases (80.6%),the etiology were found; Biliary Atresia(BA) (38.8%) and CMV hepatitis (37.3%) were the leading etiology in this group of patients of infantile cholestasis; Congenital high direct hyperbilirubinemia, Galactosemias, Intrahepatic interlobular duct absent are 1 case each; but still 13(19.4%)cases were not definite.2. There were significantly difference in the level of Y-GT between BA group and non BA group (P<0.01); 3. liver cellular swelling, cholestasis, fibroplasia and bile duct proliferation are the most common pathological changes in these infantile cholestasis cellular neorobiosis and extramedullary hemopoiesis are not common. The main changes of BA are bile duct proliferation, fibroplasia, liver cellular swelling and cholestasis, bile duct proliferation have good sensitivity (85%) and specificity (88%), cellular neorobiosis and extramedullary hemopoiesis are not exist in BA。Liver cellular swelling, lymphocyte infiltration, cholestasis and multinucleated giant cells are common in non-BA cases, but not a single feature has good sensitivity and specificity both。4.by the scoring system in the interpretation of histology in infantile cholestasis devised by Way Seah Lee, sensitivity of BA was 88.5%, and specificity was 90.2%, the positive Predictive value (PV) was 85.2%, negative PV was 92.5%. Conclusions:BA and CMV hepatitis are the most common cause of infantile cholestasis with unknown etiology or inefficiency after clinical treatment; the scoring system in the interpretation of histology in infantile cholestasis andγ-GT play an important role of differing the BA from non-BA diseases in infantile cholestasis.