title: Hereditary pancreatitis: endoscopic and surgical management.
abstract: BACKGROUND
  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.
authors: Department of Surgery, Indiana University Medical Center, Indianapolis, IN 46202, USA. eceppa@iupui.edu
authors: Eugene P
authors: EP
authors: Ceppa
authors: Henry A
authors: HA
authors: Pitt
authors: JoAnna L
authors: JL
authors: Hunter
authors: Charles M
authors: CM
authors: Leys
authors: Nicholas J
authors: NJ
authors: Zyromski
authors: Frederick J
authors: FJ
authors: Rescorla
authors: Kumar
authors: K
authors: Sandrasegaran
authors: Evan L
authors: EL
authors: Fogel
authors: Lee W
authors: LW
authors: McHenry
authors: James L
authors: JL
authors: Watkins
authors: Stuart
authors: S
authors: Sherman
authors: Glen A
authors: GA
authors: Lehman
mesh_terms: D000293:Adolescent; D000328:Adult; D002352:Carrier Proteins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D002760:Cholangiopancreatography, Endoscopic Retrograde; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D004322:Drainage; D004724:Endoscopy / Q000379:methods*; D019160:Endosonography; D005820:Genetic Testing; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009154:Mutation; D010190:Pancreatic Neoplasms / Q000175:diagnosis; D010195:Pancreatitis / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics* / Q000601:surgery*; D017410:Practice Guidelines as Topic; D012008:Recurrence; D012189:Retrospective Studies; D018709:Statistics, Nonparametric; D015607:Stents; D014057:Tomography, X-Ray Computed; D014357:Trypsin / Q000235:genetics; D014359:Trypsin Inhibitor, Kazal Pancreatic; D014481:United States / Q000453:epidemiology
chemical_list: C505032:CFTR protein, human; D002352:Carrier Proteins; C486642:SPINK1 protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D014359:Trypsin Inhibitor, Kazal Pancreatic; C489303:PRSS1 protein, human; D014357:Trypsin