Recurrent idiopathic pancreatitis in familial adenomatous polyposis: Report of a case-series and review of the literatureReport as inadecuate




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Familial Cancer

, Volume 6, Issue 3, pp 275–280

First Online: 21 February 2007Received: 17 December 2006Accepted: 24 January 2007DOI: 10.1007-s10689-007-9118-z

Cite this article as: van Esch, A.A.J., Drenth, J.P.H., te Morsche, R.H.M. et al. Familial Cancer 2007 6: 275. doi:10.1007-s10689-007-9118-z

Abstract

Familial adenomatous polyposis FAP is characterized by the development of multiple adenomatous polyps predominantly in the colon but also in the duodenum. Scattered case reports indicate that there is a risk for pancreatitis in FAP. The most likely cause of pancreatitis in FAP is obstructing ampullary adenomas. We describe 7 FAP patients who experienced one or more episodes of pancreatitis. Two patients experienced pancreatitis after endoscopic treatment of ampullary adenoma. The cause of the pancreatitis in 5 of 7 patients could not be determined, as none of the patients had obstruction of the ampulla. Furthermore, other risk factors for pancreatitis such as pancreatic serine protease inhibitor Kazal type I SPINK1 gene mutations were ruled out. A review of literature identified 20 FAP patients who developed the first episode of pancreatitis at a mean age of 45 years range 23–72 years. Some 55% had recurrent episodes of pancreatitis. Eight patients had peri ampullary adenomas or carcinomas. In most cases, the course of pancreatitis was mild with an uneventful outcome, but one patient died after an episode of acute pancreatitis.

KeywordsFamilial adenomatous polyposis Pancreatitis SPINK1 Grant support: Joost P.H. Drenth is a recipient of a NWO-VIDI grant

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Author: Aura A. J. van Esch - Joost P. H. Drenth - Rene H. M. te Morsche - Jan B. M. J. Jansen - Fokko M. Nagengast

Source: https://link.springer.com/



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