DEGENERATION OF INTRAHEPATIC BILE-DUCT WITH LYMPHOCYTE INFILTRATION INTO BILIARY EPITHELIAL-CELLS IN BILIARY ATRESIA

Degenerative changes of intrahepatic bile ducts with lymphocytic infil tration into portal areas have been recognized as the characteristic h istopathologic features in the liver with biliary atresia (BA). Occasi onally, lymphocytic infiltration into biliary ductal epithelium was no ted and has been thought to be mostly the result of nonspecific chroni c inflammation. However, this histological presentation looks quite si milar to that of acute graft-versus host disease (GVHD) after bone mar row transplantation (BMT) or acute cellular rejection (ACR) after orth otopic liver transplantation (OLTx). According to this histological si milarity, we presumed that the degeneration of intrahepatic bile ducts (DIED) might have developed with lymphocytic infiltration into biliar y epithelial cells (LIBE) as some part of an immunologic reaction, and focused on the DIED with LIBE in the patients with BA. Liver specimen s obtained from 31 patients with BA at the time of primary Kasai opera tion and 3 patients at the time of reoperation were reviewed histologi cally, and investigated DIED with LIBE. Nine patients with choledochal cyst (CBD) and 5 patients with neonatal hepatitis (NH) were added to this study as a control population. All patients with BA presented wit h DIED with LIRE. On the other hand, only 2 patients with CBD presente d with DIED with LIBE, and no patients with NH presented with DIED wit h LIBE. The DIED with LIBE is not limited to patients with BA, but it seems to be much more serious in this group. These findings may sugges t that some sort of immunologic factors contribute to the persistent D IED in the patients with BA. Presumably, the suppression of lymphocyti c activity could be an important key to further improvement in the out come of BA. Copyright (C) 1995 by W.B. Saunders Company