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Aspects of biliary atresia and other pediatric cholestatic diseases

(2009) Yang, Huiqi

In this test book several aspects of biliary atresia and other cholestatic diseases in childhood were studied. Part I aimed at the pathogenesis of biliary atresia. It used an animal model in which newborn mice developed to biliary atresia as a result of infection with rotavirus. First of all, an overview of this model was given. Then, it showed that liver fibrosis started already at an early stage by using this animal model. The liver fibrosis and epithelial-to-mesenchymal transition were accompanied by the activation of Hedgehog signal transduction. The liver fibrosis happened already before biliary obstruction. Afterwards the impact of inflammation on hepatobiliary transport system was studied. In this animal model, rotavirus infected the cholangiocyte.. Before biliary obstruction, inflammatory response arose. Down-regulation of hepatobiliary transporters leaded to early intrahepatic cholestasis. This is possibly the reason for the early initiation of liver fibrosis. In part II, clinical aspects of biliary atresia and other cholestatic diseases were studied. In the first chapter the long-term results of partial external biliary diversion were studied at children with progressive family intrahepatic cholestasis and the syndrome of Alagille. This treatment appeared to be successful at children without serious liver fibrosis in pre-operative biopsy. From meta-analysis, it seemed that children of biliary atresia might benefit from high dose steroids after operation. A retrospective study in children with biliary atresia finally showed that macroscopically visible biliary discharge during the operation at children who underwent Kasai was a favorable prognostic factor.

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