Abstract

Hereditary pancreatitis (HP)is an autosomal dominant disorder with incomplete penetrance. It is characterized by recurrent episodes of severe abdominal pain which often presents in early childhood. A mutation in the cationic trypsinogen gene has been identified in this disease. Medical management of HP at times may be different. In non-hereditary pancreatitis, the approach to management is tailored towards the individual etiology, (sphincter of Oddi dysfunction, common bile duct stones, ect.) Aim: To determine if ERCP therapeutic intervention has any role in resolving the frequency of pain in HP. Methods: We have evaluated, retrospectively using a computer data base retrieval system, a series of nine pts who had documented, by history, familial pancreatitis. All of the pts reported pain or recurrent abdominal pain on initial presentation. They reported multiple hospitalizations prior to the initial presentation per acute pancreatitis. Five of 9 pts had pancreatic duct (PD) stone. Four out of 9 pts had a PD stricture. Two of 9 had both PD stricture and stones. Two of 9 pts had no stones or strictures, but did have blunting of the terminal branches of the PD, consistent with chronic pancreatitis. Results: ERCP outlined the PD in all pts, Only those pts with PD stones or PD stricture had improvement with either endoscopic sphincterotomy, PD stent placement or stone retrieval. Two of 9 pts had no PD stones or strictures and did not have any symptomatic improvement with ERCP intervention as noted by no significant change in frequency of abdominal pain or hospitalizations. Conclusion: Preliminary results have shown that a therapeutic ERCP is only helpful when there are structural defects such as pancreatic duct strictures or pancreatic duct stones. In the absence of such structural defects therapeutic intervention may have little benefit. 5295

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