Abstract
A 15-year-old with Kabuki syndrome and cytochrome C deficiency presented with recurrent acute pancreatitis. Laboratory results included alkaline phosphatase 397, alanine aminotransferase 90, aspartate aminotransferase 43, TB 0.7. MRI revealed a dilatated biliary tree with abrupt distal tapering that was concerning for stricture and nonvisualization of the pancreatic duct in the body, with upstream dilatation and irregularity (A). The pancreaticobiliary junction (PBJ) could not be delineated.
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