Recurrent idiopathic pancreatitis in familial adenomatous polyposis: report of a case-series and review of the literature.
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.
van Esch
Aura A J
AA
Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Center, PO Box 9101, 6500 HB Nijmegen, The Netherlands. A.vanEsch@MDL.umcn.nl
Drenth
Joost P H
JP
te Morsche
Rene H M
RH
Jansen
Jan B M J
JB
Nagengast
Fokko M
FM
D011125:Adenomatous Polyposis Coli / Q000150:complications* / Q000235:genetics; D000293:Adolescent; D000328:Adult; D002352:Carrier Proteins / Q000235:genetics; D004379:Duodenal Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D050500:Pancreatitis, Chronic / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D011127:Polyps / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D012008:Recurrence; D012307:Risk Factors; D014359:Trypsin Inhibitor, Kazal Pancreatic
D002352:Carrier Proteins; C486642:SPINK1 protein, human; D014359:Trypsin Inhibitor, Kazal Pancreatic