| Co-Investigator(Kenkyū-buntansha) |
SUZUKI Tetsuya University of Yamanashi, Faculty of Medicine, Research Associate, 医学部, 助手 (30324198)
ITAKURA Jun University of Yamanashi, Faculty of Medicine, Research Associate, 医学部, 助手 (10252032)
FUJII Hideki University of Yamanashi, Faculty of Medicine, Associate Professor, 医学部, 助教授 (30181316)
IKEDA Seiyo Fukuoka University, School of Medicine, Professor, 医学部, 教授 (40038758)
SUDA Koich Juntendo University, School of Medicine, Professor, 医学部, 教授 (80090596)
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| Research Abstract |
Pancreaticobiliary maljunction (PBM), a congenital anomaly defined as the union of the pancreatic and biliary ducts located outside the duodenal wall, is a recently designated disease entity, and is frequently associated with congenital choledochal cyst (CCBD), which is thought to be different from PBM in embryonic etiology, and variegated clinical features, such as digestive diseases appear in patients with PBM and CCBD.
From an analysis of 250 PBM patients (169 with benign hepatobiliary and pancreatic disease and 81 with malignancy) the pathophysiologic and clinical aspects and biliary carcinogenesis in PBM patients were reviewed. PBM is thought to develop as a misarrangement of the embryonic connections in the pancreaticobiliary ductal system, in which the terminal bile duct is joined with one of the ducts of the ventral pancreas.
The clinical aspects are intermittent abdominal pain, relapsing acute pancreatitis, jaundice, cholangitis and gallbladder cancer. In patients with PBM and C
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CBD, primary bile duct stones, acute cholangitis and bile duct cancer are considered to result from cholestasis, regurgitation of pancreatic juice, and reciprocal reflux of bile and pancreatic juice. Moreover, the mixture of bile and pancreatic juice due to reciprocal reflux very likely plays an important role in biliary carcinogenesis.
In PBM patients, an elevation of cellular proliferation activity in the gallbladder epithelia, was reported. A number of oncogenes and tumor suppressor genes have been identified and implicated in carcinogenesis, and, in particular, the K-ras oncogene and the p53 suppressor gene are the best-studied genes in gallbladder carcinogenesis with PBM. K-ras mutations were reported that did not appear essential for hyperplasia, but may be an early event in carcinogenesis. The p53 mutations were also reported to be involved in carcinogenesis in the biliary epithelium in PBM patients.
Key Words: misarrangement of the embryonic connections in pacreaticobiliary ductal system, gallbladder cancer, bile duct cancer, relapsing acute pancreatitis, obstructive jaundice. Less
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