Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Causes of chronic pancreatitis in children and adolescents

Mohamad Miqdady, MD
Seiji Kitagawa, MD
Section Editors
William J Klish, MD
David C Whitcomb, MD, PhD
Deputy Editor
Alison G Hoppin, MD


Chronic pancreatitis is a progressive inflammatory disease of the pancreas. It is characterized by structural changes that result in irreversible exocrine and/or endocrine pancreatic insufficiency [1-3]. The structural changes include diffuse or focal destruction, sclerosis, acinar cell loss, islet cell loss, inflammatory cell infiltrate, and pancreatic duct abnormalities. Intraductal obstruction may be caused by protein-plugs and/or calculi.

Because the pancreas has a large reserve capacity, symptoms of pancreatic failure do not develop until there is loss of over 90 percent of pancreatic exocrine function [4]. Most patients with chronic pancreatitis initially experience recurrent abdominal pain, which may progress to persistent pain. Less commonly, patients are asymptomatic or have minimal symptoms until developing signs of advanced disease, such as diabetes mellitus or fat and protein malabsorption. With advances in diagnostic methods, the diagnosis of chronic pancreatitis is becoming more common in children. (See "Clinical manifestations and diagnosis of chronic pancreatitis in children".)

A variety of pathologic processes can cause chronic pancreatitis in children. However, no specific etiology can be identified for many cases, termed "idiopathic chronic pancreatitis." Hereditary pancreatitis is a form of chronic pancreatitis that often presents during childhood and adolescence; it will be discussed briefly here and in detail in a separate topic review. (See "Hereditary pancreatitis".)

The following issues are discussed in separate topic reviews:

The clinical manifestations and diagnosis of chronic pancreatitis in children. (See "Clinical manifestations and diagnosis of chronic pancreatitis in children".)


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Jul 2016. | This topic last updated: Sep 24, 2015.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
  1. Owyang C. Chronic pancreatitis. In: Textbook of Gastroenterology, 4th, Yamada T (Ed), Lippincott, Williams and Wilkins, Philadelphia 2003. p.2061.
  2. Sarner M. Pancreatitis definition and classification. In: The Pancreas: Pathology and Disease, 2nd, Go VL, DiMagno EP, Gardner JD, Lebenthal E, et al (Eds), Raven, New York 1993. p.575.
  3. Steer ML, Waxman I, Freedman S. Chronic pancreatitis. N Engl J Med 1995; 332:1482.
  4. Gaskin KJ, Durie PR, Lee L, et al. Colipase and lipase secretion in childhood-onset pancreatic insufficiency. Delineation of patients with steatorrhea secondary to relative colipase deficiency. Gastroenterology 1984; 86:1.
  5. Etemad B, Whitcomb DC. Chronic pancreatitis: diagnosis, classification, and new genetic developments. Gastroenterology 2001; 120:682.
  6. Clifton MS, Pelayo JC, Cortes RA, et al. Surgical treatment of childhood recurrent pancreatitis. J Pediatr Surg 2007; 42:1203.
  7. Schwarzenberg SJ, Bellin M, Husain SZ, et al. Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden. J Pediatr 2015; 166:890.
  8. Whitcomb DC, Lowe ME. Pancreatitis: Acute and chronic. In: Walker's Pediatric Gastrointestinal Disease, Kleinman RE, Sanderson IR, Goulet O-J, et al (Eds), BC Decker, Inc., Ontario 2008. Vol 2, p.1213.
  9. Mehta DI. Acute and chronic pancreatitis in childhood. Indian J Pediatr 1999; 66:S81.
  10. Slakey DP, Johnson CP, Cziperle DJ, et al. Management of severe pancreatitis in renal transplant recipients. Ann Surg 1997; 225:217.
  11. Corrodi P, Knoblauch M, Binswanger U, et al. Pancreatitis after renal transplantation. Gut 1975; 16:285.
  12. Cho MH, Hong EH, Lee HS, Ko CW. Recurrent pancreatitis after renal transplantation in a child. Pediatr Transplant 2008; 12:593.
  13. Mathew P, Wyllie R, Caulfield M, et al. Chronic pancreatitis in late childhood and adolescence. Clin Pediatr (Phila) 1994; 33:88.
  14. Wang W, Liao Z, Li ZS, et al. Chronic pancreatitis in Chinese children: etiology, clinical presentation and imaging diagnosis. J Gastroenterol Hepatol 2009; 24:1862.
  15. Witt H, Luck W, Hennies HC, et al. Mutations in the gene encoding the serine protease inhibitor, Kazal type 1 are associated with chronic pancreatitis. Nat Genet 2000; 25:213.
  16. Pfützer RH, Barmada MM, Brunskill AP, et al. SPINK1/PSTI polymorphisms act as disease modifiers in familial and idiopathic chronic pancreatitis. Gastroenterology 2000; 119:615.
  17. Layer P, Yamamoto H, Kalthoff L, et al. The different courses of early- and late-onset idiopathic and alcoholic chronic pancreatitis. Gastroenterology 1994; 107:1481.
  18. Pitchumoni CS, Mohan V.. Juvenile tropical pancreatitis. In: Pediatric Gastrointestinal Disease, 4 E, Walker WA, Goulet O, Kleinman RE, et al.. (Eds), BC Decker, Ontario 2004. Vol 2, p.1598-1605.
  19. Sarles H, Augustine P, Laugier R, et al. Pancreatic lesions and modifications of pancreatic juice in tropical chronic pancreatitis (tropical calcific diabetes). Dig Dis Sci 1994; 39:1337.
  20. Bhatia E, Choudhuri G, Sikora SS, et al. Tropical calcific pancreatitis: strong association with SPINK1 trypsin inhibitor mutations. Gastroenterology 2002; 123:1020.
  21. Schneider A, Suman A, Rossi L, et al. SPINK1/PSTI mutations are associated with tropical pancreatitis and type II diabetes mellitus in Bangladesh. Gastroenterology 2002; 123:1026.
  22. Sultan M, Werlin S, Venkatasubramani N. Genetic prevalence and characteristics in children with recurrent pancreatitis. J Pediatr Gastroenterol Nutr 2012; 54:645.
  23. Creighton J, Lyall R, Wilson DI, et al. Mutations of the cationic trypsinogen gene in patients with chronic pancreatitis. Lancet 1999; 354:42.
  24. Witt H, Luck W, Becker M. A signal peptide cleavage site mutation in the cationic trypsinogen gene is strongly associated with chronic pancreatitis. Gastroenterology 1999; 117:7.
  25. Fink EN, Kant JA, Whitcomb DC. Genetic counseling for nonsyndromic pancreatitis. Gastroenterol Clin North Am 2007; 36:325.
  26. Masson E, Chen JM, Scotet V, et al. Association of rare chymotrypsinogen C (CTRC) gene variations in patients with idiopathic chronic pancreatitis. Hum Genet 2008; 123:83.
  27. Keim V, Bauer N, Teich N, et al. Clinical characterization of patients with hereditary pancreatitis and mutations in the cationic trypsinogen gene. Am J Med 2001; 111:622.
  28. Sossenheimer MJ, Aston CE, Preston RA, et al. Clinical characteristics of hereditary pancreatitis in a large family, based on high-risk haplotype. The Midwest Multicenter Pancreatic Study Group (MMPSG). Am J Gastroenterol 1997; 92:1113.
  29. Whitcomb DC. Genetic predispositions to acute and chronic pancreatitis. Med Clin North Am 2000; 84:531.
  30. Sibert JR. Hereditary pancreatitis in England and Wales. J Med Genet 1978; 15:189.
  31. Howes N, Lerch MM, Greenhalf W, et al. Clinical and genetic characteristics of hereditary pancreatitis in Europe. Clin Gastroenterol Hepatol 2004; 2:252.
  32. Gargouri L, Ponsot P, Viala J, et al. Recurrent autoimmune pancreatitis in a 10-year-old boy. J Pediatr Gastroenterol Nutr 2009; 48:374.
  33. Fukumori K, Shakado S, Miyahara T, et al. Atypical manifestations of pancreatitis with autoimmune phenomenon in an adolescent female. Intern Med 2005; 44:886.
  34. Ectors N, Maillet B, Aerts R, et al. Non-alcoholic duct destructive chronic pancreatitis. Gut 1997; 41:263.
  35. Fujii LL, Chari ST, El-Youssef M, et al. Pediatric pancreatic EUS-guided trucut biopsy for evaluation of autoimmune pancreatitis. Gastrointest Endosc 2013; 77:824.
  36. Kamisawa T, Yoshiike M, Egawa N, et al. Treating patients with autoimmune pancreatitis: results from a long-term follow-up study. Pancreatology 2005; 5:234.
  37. Okazaki K, Uchida K, Matsushita M, Takaoka M. Autoimmune pancreatitis. Intern Med 2005; 44:1215.
  38. Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001; 344:732.
  39. Okazaki K, Chiba T. Autoimmune related pancreatitis. Gut 2002; 51:1.
  40. Meltzer SJ, Korelitz BI. Pancreatitis and duodenopancreatic reflux in Crohn's disease. Case report and review of the literature. J Clin Gastroenterol 1988; 10:555.
  41. Newman LH, Wellinger JR, Present DH, Aufses AH Jr. Crohn's disease of the duodenum associated with pancreatitis: a case report and review of the literature. Mt Sinai J Med 1987; 54:429.
  42. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 3-1994. An 11-year-old boy with recurrent abdominal pain and evidence of pancreatitis. N Engl J Med 1994; 330:196.
  43. Broide E, Dotan I, Weiss B, et al. Idiopathic pancreatitis preceding the diagnosis of inflammatory bowel disease is more frequent in pediatric patients. J Pediatr Gastroenterol Nutr 2011; 52:714.
  44. Gorry MC, Gabbaizedeh D, Furey W, et al. Mutations in the cationic trypsinogen gene are associated with recurrent acute and chronic pancreatitis. Gastroenterology 1997; 113:1063.
  45. Lewin-Smith MR, Dipalma JS, Hoy GR, et al. Chronic fibrosing pancreatitis in childhood: report of a case and literature review. Pediatr Pathol Lab Med 1996; 16:681.
  46. Elitsur Y, Siddiqui SY, Sloven D, et al. Chronic pancreatitis with diffuse fibrosis in early childhood. Pancreas 1989; 4:504.
  47. Kamelmaz I, Elitsur Y. Pancreas divisum--the role of ERCP in children. W V Med J 1999; 95:14.
  48. Guelrud M, Morera C, Rodriguez M, et al. Normal and anomalous pancreaticobiliary union in children and adolescents. Gastrointest Endosc 1999; 50:189.
  49. Koc-Kuś J. [Contemporary opinions on the etiology of chronic pancreatitis]. Wiad Lek 1999; 52:289.
  50. Kamisawa T, Egawa N, Tsuruta K, et al. Pancreatitis associated with congenital abnormalities of the pancreaticobiliary system. Hepatogastroenterology 2005; 52:223.
  51. Amerson JL, Ricketts RR. Idiopathic fibrosing pancreatitis: a rare cause of obstructive jaundice in children. Am Surg 1996; 62:295.
  52. Meneely RL, O'Neill J, Ghishan FK. Fibrosing pancreatitis--an obscure causes of painless obstructive jaundice: a case report and review of the literature. Pediatrics 1981; 67:136.
  53. Harb R, Naon H. Idiopathic fibrosing pancreatitis in a 3-year-old girl: a case report and review of the literature. J Pediatr Surg 2005; 40:1335.
  54. Buxbaum JL, Eloubeidi MA, Varadarajulu S. Utility of EUS-guided FNA in the management of children with idiopathic fibrosing pancreatitis. J Pediatr Gastroenterol Nutr 2011; 52:482.
  55. Sylvester FA, Shuckett B, Cutz E, et al. Management of fibrosing pancreatitis in children presenting with obstructive jaundice. Gut 1998; 43:715.
  56. El-Matary W, Casson D, Hodges S, et al. Successful conservative management of idiopathic fibrosing pancreatitis in children. Eur J Pediatr 2006; 165:560.