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.
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.
Authors: M Schenk; A G Schwartz; E O'Neal; M Kinnard; J K Greenson; J P Fryzek; G S Ying; D H Garabrant Journal: J Natl Cancer Inst Date: 2001-04-18 Impact factor: 13.506
Authors: Syed A Ahmad; Andrew M Lowy; Curtis J Wray; David D'Alessio; Kywran A Choe; Laura E James; Andreas Gelrud; Jeffrey B Matthews; Horacio L R Rilo Journal: J Am Coll Surg Date: 2005-09-23 Impact factor: 6.113
Authors: David E R Sutherland; David M Radosevich; Melena D Bellin; Bernard J Hering; Gregory J Beilman; Ty B Dunn; Srinath Chinnakotla; Selwyn M Vickers; Barbara Bland; A N Balamurugan; Martin L Freeman; Timothy L Pruett Journal: J Am Coll Surg Date: 2012-03-06 Impact factor: 6.113
Authors: D C Whitcomb; R A Preston; C E Aston; M J Sossenheimer; P S Barua; Y Zhang; A Wong-Chong; G J White; P G Wood; L K Gates; C Ulrich; S P Martin; J C Post; G D Ehrlich Journal: Gastroenterology Date: 1996-06 Impact factor: 22.682
Authors: A Sojo Aguirre; N Martínez Ezquerra; C Bousoño García; M D García Novo; S Heredia González; J Manzanares López-Manzanares; F Baranda García; C Vázquez Cordero Journal: An Pediatr (Barc) Date: 2011-08-04 Impact factor: 1.500
Authors: J W Poley; I Kluijt; D J Gouma; F Harinck; A Wagner; C Aalfs; C H J van Eijck; A Cats; E J Kuipers; Y Nio; P Fockens; M J Bruno Journal: Am J Gastroenterol Date: 2009-06-02 Impact factor: 10.864
Authors: Srinath Chinnakotla; David M Radosevich; Ty B Dunn; Melena D Bellin; Martin L Freeman; Sarah J Schwarzenberg; A N Balamurugan; Josh Wilhelm; Barbara Bland; Selwyn M Vickers; Gregory J Beilman; David E R Sutherland; Timothy L Pruett Journal: J Am Coll Surg Date: 2014-01-10 Impact factor: 6.113
Authors: Maria G Sacco Casamassima; Seth D Goldstein; Jingyan Yang; Colin D Gause; Fizan Abdullah; Avner Meoded; Martin A Makary; Paul M Colombani Journal: Pediatr Surg Int Date: 2016-11-04 Impact factor: 1.827
Authors: David M Troendle; Douglas S Fishman; Bradley A Barth; Matthew J Giefer; Tom K Lin; Quin Y Liu; Maisam Abu-El-Haija; Melena D Bellin; Peter R Durie; Steven D Freedman; Cheryl Gariepy; Tanja Gonska; Melvin B Heyman; Ryan Himes; Sohail Z Husain; Soma Kumar; Mark E Lowe; Veronique D Morinville; Chee Y Ooi; Joseph Palermo; John F Pohl; Sarah Jane Schwarzenberg; Steven Werlin; Michael Wilschanski; M Bridget Zimmerman; Aliye Uc Journal: Pancreas Date: 2017-07 Impact factor: 3.327
Authors: Lianne Scholten; Anouk Ej Latenstein; Cora M Aalfs; Marco J Bruno; Olivier R Busch; Bert A Bonsing; Bas Groot Koerkamp; I Quintus Molenaar; Dirk T Ubbink; Jeanin E van Hooft; Paul Fockens; Jolanda Glas; J Hans DeVries; Marc G Besselink Journal: United European Gastroenterol J Date: 2020-07-23 Impact factor: 4.623
Authors: Mark J Ferreira; Lindsay B McKenna; Jia Zhang; Maximilian Reichert; Basil Bakir; Elizabeth L Buza; Emma E Furth; Clifford W Bogue; Anil K Rustgi; Klaus H Kaestner Journal: Cell Mol Gastroenterol Hepatol Date: 2015-09-01