Hereditary Pancreatitis: Endoscopic and Surgical Management

Eugene P. Ceppa, Henry A. Pitt, JoAnna L. Hunter, Charles M. Leys, Nicholas J. Zyromski, Frederick J. Rescorla, Kumar Sandrasegaran, Evan L. Fogel, Lee W. McHenry, James L. Watkins, Stuart Sherman, Glen A. Lehman

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)847-857
Number of pages11
JournalJournal of Gastrointestinal Surgery
Volume17
Issue number5
DOIs
StatePublished - 2013
Externally publishedYes

Fingerprint

Chronic Pancreatitis
Pancreatic Neoplasms
Genetic Databases
Mutation
Pancreatic Ducts
Genetic Testing
Hereditary pancreatitis
Cystic Fibrosis
Pancreatitis
Drainage
Young Adult
Anatomy
Guidelines
Pediatrics
Pain
Therapeutics

Keywords

  • Acute pancreatitis
  • CFTR
  • Chronic pancreatitis
  • Endoscopic retrograde cholangiopancreatography
  • Endoscopic ultrasound surgery
  • Endotherapy
  • Hereditary pancreatitis
  • PRSS1
  • SPINK1

ASJC Scopus subject areas

  • Surgery
  • Gastroenterology

Cite this

Ceppa, E. P., Pitt, H. A., Hunter, J. L., Leys, C. M., Zyromski, N. J., Rescorla, F. J., ... Lehman, G. A. (2013). Hereditary Pancreatitis: Endoscopic and Surgical Management. Journal of Gastrointestinal Surgery, 17(5), 847-857. https://doi.org/10.1007/s11605-013-2167-8

Hereditary Pancreatitis : Endoscopic and Surgical Management. / Ceppa, Eugene P.; Pitt, Henry A.; Hunter, JoAnna L.; Leys, Charles M.; Zyromski, Nicholas J.; Rescorla, Frederick J.; Sandrasegaran, Kumar; Fogel, Evan L.; McHenry, Lee W.; Watkins, James L.; Sherman, Stuart; Lehman, Glen A.

In: Journal of Gastrointestinal Surgery, Vol. 17, No. 5, 2013, p. 847-857.

Research output: Contribution to journalArticle

Ceppa, EP, Pitt, HA, Hunter, JL, Leys, CM, Zyromski, NJ, Rescorla, FJ, Sandrasegaran, K, Fogel, EL, McHenry, LW, Watkins, JL, Sherman, S & Lehman, GA 2013, 'Hereditary Pancreatitis: Endoscopic and Surgical Management', Journal of Gastrointestinal Surgery, vol. 17, no. 5, pp. 847-857. https://doi.org/10.1007/s11605-013-2167-8
Ceppa EP, Pitt HA, Hunter JL, Leys CM, Zyromski NJ, Rescorla FJ et al. Hereditary Pancreatitis: Endoscopic and Surgical Management. Journal of Gastrointestinal Surgery. 2013;17(5):847-857. https://doi.org/10.1007/s11605-013-2167-8
Ceppa, Eugene P. ; Pitt, Henry A. ; Hunter, JoAnna L. ; Leys, Charles M. ; Zyromski, Nicholas J. ; Rescorla, Frederick J. ; Sandrasegaran, Kumar ; Fogel, Evan L. ; McHenry, Lee W. ; Watkins, James L. ; Sherman, Stuart ; Lehman, Glen A. / Hereditary Pancreatitis : Endoscopic and Surgical Management. In: Journal of Gastrointestinal Surgery. 2013 ; Vol. 17, No. 5. pp. 847-857.
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abstract = "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.",
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AU - Pitt, Henry A.

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AU - Leys, Charles M.

AU - Zyromski, Nicholas J.

AU - Rescorla, Frederick J.

AU - Sandrasegaran, Kumar

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AU - Sherman, Stuart

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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.

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