关键词: 七鳃鳗, 变态发育, 胆道闭锁, 胆汁淤积, 耐受机制

Abstract:

Biliary atresia (BA) is a rare biliary disease in infants and young children, which is characterized by fibrosclerotic bile duct disease, leading to extrahepatic and intrahepatic bile duct obstruction or occlusion. The bile acids cannot be excreted to the intestinal tract, which causes serious damage to the liver parenchyma cells, and eventually leads to life-threatening cirrhosis and chronic liver failure. At present, the pathogenesis of biliary atresia is unknown. The sequential treatment of “first Gexi” and “later transplantation” is nowadays widely adopted in the clinic. Gossi operation (Kasai) can prolong the survival time of autologous liver by the establishment of bile drainage channel. However, it is necessary for patients to perform liver transplantation with the emergence of complications such as portal hypertension and primary cholangitis. Lampreys are the only vertebrates whose bile duct can disappear spontaneously in the process of growth and development. Lampreys have complete biliary system at the larval stage, but in the process of metamorphosis, lampreys show developmental biliary atresia, bile duct and gallbladder gradually degenerate until the whole biliary system is completely lost, and hepatocytes undergo rearrangement and fine structural changes at the same time. It has been found that lampreys can maintain normal plasma bile acid levels when the symptoms of biliary atresia are formed in during metamorphosis, so that liver cirrhosis and hepatic failure do not occur, thereby adapting to and surviving biliary atresia and cholestasis. To explore the application of bile acids tolerance in biliary atresia, we summarize the recent research progress on the mechanism of bile acids tolerance caused by biliary atresia in lampreys, and further provide a reference for the development of diagnosis and treatments of human biliary atresia.

Key words: lampreys, metamorphosis, biliary atresia, cholestasis, tolerance mechanism