Abstract
Pancreaticobiliary maljunction (PBM) causes pancreaticobiliary reflux. Regurgitated pancreatic juice contains trypsinogen and lithostathine. Activated trypsin cleaves soluble lithostathine into insoluble forms, which aggregate to protein plugs. Protein plugs obstruct the common channel or the narrow segment and produce characteristically intermittent symptoms and signs in most of pediatric patients. These include abdominal pain, vomiting, jaundice, and elevated levels of serum transaminases and amylase. Most plugs are fragile and disappear spontaneously; however, plugs are produced repeatedly, which explains why symptoms are usually mild and self-limiting but also why they recur. Exceptions to this protein plug theory include congenital biliary dilatation with neonatal and early infant onset. In these neonates and infants, the extremely narrow segment causes obstructive cholangiopathy independent of reflux. The mixture of bile and regurgitated pancreatic juice produces substances hazardous to the biliary epithelium. The resulting chronic inflammation causes multistep carcinogenesis through a hyperplasia-dysplasia-carcinoma sequence.
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Kaneko, K. (2018). Pathophysiology of Pancreaticobiliary Maljunction and Congenital Biliary Dilatation. In: Kamisawa, T., Ando, H. (eds) Pancreaticobiliary Maljunction and Congenital Biliary Dilatation. Springer, Singapore. https://doi.org/10.1007/978-981-10-8654-0_8
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DOI: https://doi.org/10.1007/978-981-10-8654-0_8
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