Pancreaticobiliary maljunction (PBM) is a congenital anomaly in which the reciprocal reflux between pancreatic and bile juice occurs because the conflu- ence of the pancreatic and bile ducts lies outside of the area of contractile influence of the sphincter of Oddi. The reflux of pancreatic juice into the bile duct and subse- quent elevated pancreatic enzyme level in bile are usually recognized in PBM. Amylase is the most popular pancreatic enzyme for assessment in daily prac- tice, and assessment of amylase level in bile is useful to definitively confirm the presence of PBM, and the mixture of pancreatic and bile juices may produce various physiological and pathological alterations. In PBM, the amylase level in bile in patients with biliary cancers has been reported to be higher than that with benign biliary diseases; however, the relationship between the amylase level in bile and development of biliary diseases has not been well documented. Recently, several studies have demonstrated that some patients with a normal morphological pancre- aticobiliary junction have a high amylase level and biliary carcinoma. This suggests the possibility that pancreaticobiliary reflux can occur even in patients without PBM and result in the development of biliary carcinoma in the same manner as in patients with PBM.
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