TY - JOUR
T1 - Hereditary Pancreatitis
T2 - Endoscopic and Surgical Management
AU - Ceppa, Eugene P.
AU - Pitt, Henry A.
AU - Hunter, Jo Anna L.
AU - Leys, Charles M.
AU - Zyromski, Nicholas J.
AU - Rescorla, Frederick J.
AU - Sandrasegaran, Kumar
AU - Fogel, Evan L.
AU - McHenry, Lee W.
AU - Watkins, James L.
AU - Sherman, Stuart
AU - Lehman, Glen A.
N1 - Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2013/5
Y1 - 2013/5
N2 - Introduction: Hereditary pancreatitis is a rare cause of chronic pancreatitis. In recent years, genetic mutations have been characterized. The rarity of this disorder has resulted in a gap in clinical knowledge. The aims were to characterize patients with hereditary pancreatitis and establish clinical guidelines. Methods: Pediatric and adult endoscopic, surgical, radiologic, and genetic databases from 1998 to 2012 were searched. Patients with recurrent acute or chronic pancreatitis and genetic mutation for either PRSS-1, SPINK-1, or CFTR or those who met the family history criteria were included. Patients with pancreatitis due to other causes, without a positive family history, familial pancreatic cancer, or cystic fibrosis, were excluded. Results: Eighty-seven patients were identified. Genetic testing confirmed the diagnosis in 54 patients (62 %). Eighty-five patients (98 %) underwent 263 endoscopic procedures including sphincterotomy (72 %), stone removal (49 %), and pancreatic duct stenting (82 %). Twenty-eight patients (32 %) have undergone 37 operations which included 19 resections and 18 drainage procedures. The interval between procedures for recurrent pain was longer for surgery than for endoscopic therapy (9. 1 vs. 3. 4 years, p < 0. 05). Conclusions: Most children and young adults with hereditary pancreatitis can be managed initially with endoscopic therapy. When surgery is undertaken, the procedure should be tailored to the pancreatic anatomy and cancer risk.
AB - Introduction: Hereditary pancreatitis is a rare cause of chronic pancreatitis. In recent years, genetic mutations have been characterized. The rarity of this disorder has resulted in a gap in clinical knowledge. The aims were to characterize patients with hereditary pancreatitis and establish clinical guidelines. Methods: Pediatric and adult endoscopic, surgical, radiologic, and genetic databases from 1998 to 2012 were searched. Patients with recurrent acute or chronic pancreatitis and genetic mutation for either PRSS-1, SPINK-1, or CFTR or those who met the family history criteria were included. Patients with pancreatitis due to other causes, without a positive family history, familial pancreatic cancer, or cystic fibrosis, were excluded. Results: Eighty-seven patients were identified. Genetic testing confirmed the diagnosis in 54 patients (62 %). Eighty-five patients (98 %) underwent 263 endoscopic procedures including sphincterotomy (72 %), stone removal (49 %), and pancreatic duct stenting (82 %). Twenty-eight patients (32 %) have undergone 37 operations which included 19 resections and 18 drainage procedures. The interval between procedures for recurrent pain was longer for surgery than for endoscopic therapy (9. 1 vs. 3. 4 years, p < 0. 05). Conclusions: Most children and young adults with hereditary pancreatitis can be managed initially with endoscopic therapy. When surgery is undertaken, the procedure should be tailored to the pancreatic anatomy and cancer risk.
KW - Acute pancreatitis
KW - CFTR
KW - Chronic pancreatitis
KW - Endoscopic retrograde cholangiopancreatography
KW - Endoscopic ultrasound surgery
KW - Endotherapy
KW - Hereditary pancreatitis
KW - PRSS1
KW - SPINK1
UR - http://www.scopus.com/inward/record.url?scp=84876137598&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84876137598&partnerID=8YFLogxK
U2 - 10.1007/s11605-013-2167-8
DO - 10.1007/s11605-013-2167-8
M3 - Article
C2 - 23435738
AN - SCOPUS:84876137598
SN - 1091-255X
VL - 17
SP - 847
EP - 857
JO - Journal of Gastrointestinal Surgery
JF - Journal of Gastrointestinal Surgery
IS - 5
ER -