CASE-REPORT - PAUCITY OF INTERLOBULAR BILE-DUCTS IN CHINESE CHILDREN

Sixteen Chinese children with cholestasis since early infancy were dia gnosed to have paucity of interlobular bile ducts (PILED) or its equiv alent. Twelve children belonged to the syndromic group of PILBD and fo ur children belonged to the non-syndromic group. A definite histologic al diagnosis of bile duct paucity was established in only two children (aged 4 and 9 months) during the first percutaneous needle biopsy. In the remaining 14 children a varying degree of bile duct destruction w as evident in the follow up percutaneous or wedge liver biopsies. The evolving changes were characterized by inflammatory infiltration near or at the ductal wall, the presence of dysmorphic ductules, the degene ration of ductal epithelia and a progressive decrease of interlobular bile ducts. Of 10 children who underwent laparotomy for definite diagn osis, kasai operation was performed in two of them. In the syndromic P ILED group, all children, including tao paired siblings, had at least three of live major clinical features. Hypoplasia of the extrahepatic biliary tree was found in five children and atresia of the extrahepati c bile duct was found in one. Three of six children studied were shown ; by polymerase chain reaction, to have cytomegalovirus infection in t he liver. This study demonstrates that bile duct paucity is a result o f progressive bile duct destruction. A definitive diagnosis is difficu lt to make ill early infancy Thus, the careful evaluation of extrahepa tic features in cholestatic children and follow-up liver biopsies are indicated. Although the pathogenetic mechanism of PILED is unknown, bi le duct destruction is the common pathway leading to paucity of bile d ucts irrespective of syndromic or non-syndromic types.