Chronic pancreatitis in children:Current knowledge in diagnosis and treatment

Year 2011, Volume: 3 Issue: 4, 1 - 14, 05.11.2011

Aliye Uc

Abstract

Chronic pancreatitis (CP) is a progressive inflammatory disorder of the pancreas characterized by irreversible morphological changes and fibrosis, leading to exocrine and endocrine pancreatic insufficiency. The prevalence of chronic pancreatitis in childhood is unknown. Most likely, idiopathic causes comprise a large percentage of children with CP. CP should be considered in children presenting with recurrent abdominal pain. Pain may be disabling, exocrine pancreatic insufficiency and diabetes may develop over time. Diagnostic tests include abdominal ultrasound, contrast-enhanced CT, MRCP, ERCP, EUS. Genetic etiologies should be investigated in children with CP of unknown cause. Medical treatment includes control of the pain, replacement therapy with pancreatic enzymes, antioxidants, insulin. Endoscopic interventions (sphincterotomy/stent placement) can be used to decompress the pancreatic duct. Surgical approaches (lateral pancreaticojejunostomy, total pancreatectomy and islet autotransplantation) are rarely used in pediatrics. Although significant advances have been made in our understanding of CP in childhood within the last decade, challenge remains to develop better diagnostic techniques, treatment modalities and improved outcomes.

Keywords

pediatric, chronic pancreatitis, etiology, diagnosis, therapy

References

• 1. Lowe ME. Pancreatitis in childhood. Curr.Gastroenterol.Rep. 2004;6:240- 246.
• 2. Lowe ME. Acute and Chronic Pancreatitis. In: Bishop WP, ed. Pediatric Practice Gastroenterology. New York: McGraw Hill, 2010:428- 440.
• 3. Joergensen M, Brusgaard K, Cruger DG, Gerdes AM, de Muckadell OB. Incidence, prevalence, etiology, and prognosis of first-time chronic pancreatitis in young patients: a nationwide cohort study. Dig Dis Sci 2010;55:2988-98.
• 4. Etemad B, Whitcomb DC. Chronic pancreatitis: diagnosis, classification, and new genetic developments. Gastroenterology 2001;120:682-707.
• 5. Lowe ME, Greer JB. Pancreatitis in children and adolescents. Curr.Gastroenterol.Rep. 2008;10:128- 135.
• 6. Witt H, Apte MV, Keim V, Wilson JS. Chronic pancreatitis: challenges and advances in pathogenesis, genetics, diagnosis, and therapy. Gastroenterology 2007;132:1557-73.
• 7. Kandula L, Whitcomb DC, Lowe ME. Genetic issues in pediatric pancreatitis. Curr.Gastroenterol.Rep. 2006;8:248-253.
• 8. Witt H, Becker M. Genetics of chronic pancreatitis. J Pediatr Gastroenterol Nutr 2002;34:125-36.
• 9. Whitcomb DC, Gorry MC, Preston RA, Furey W, Sossenheimer MJ, Ulrich CD, Martin SP, Gates LK, Jr., Amann ST, Toskes PP, Liddle R, McGrath K, Uomo G, Post JC, Ehrlich GD. Hereditary pancreatitis is caused by a mutation in the cationic trypsinogen gene. Nat Genet 1996;14:141-5.
• 10. Whitcomb DC. Hereditary pancreatitis: new insights into acute and chronic pancreatitis. Gut 1999;45:317-22.
• 11. Whitcomb DC. Genetic predispositions to acute and chronic pancreatitis. Med Clin North Am 2000;84:531-47, vii.
• 12. pplebaum-Shapiro SE, Finch R, Pfutzer RH, Hepp LA, Gates L, Amann S, Martin S, Ulrich CD, Whitcomb DC. Hereditary pancreatitis in North America: the PittsburghMidwest Multi-Center Pancreatic Study Group Study. Pancreatology. 2001;1:439-443.
• 13. Lowenfels AB, Maisonneuve P, Cavallini G, Ammann RW, Lankisch PG, Andersen JR, Dimagno EP, Andren-Sandberg A, Domellof L. Pancreatitis and the risk of pancreatic cancer. International Pancreatitis Study Group. N Engl J Med 1993;328:1433-7.
• 14. Lowenfels AB, Maisonneuve P, DiMagno EP, Elitsur Y, Gates LK, Jr., Perrault J, Whitcomb DC. Hereditary pancreatitis and the risk of pancreatic cancer. International Hereditary Pancreatitis Study Group. J Natl Cancer Inst 1997;89:442-6.
• 15. Steer ML, Waxman I, Freedman S. Chronic pancreatitis. N Engl J Med 1995;332:1482-90.
• 16. Darge K, Anupindi S. Pancreatitis and the role of US, MRCP and ERCP. Pediatr.Radiol. 2009;39 Suppl 2:S153- 7.:S153-S157.
• 17. Nydegger A, Couper RT, Oliver MR. Childhood pancreatitis. J Gastroenterol.Hepatol. 2006;21:499- 509.
• 18. Varadarajulu S, Wilcox CM, Eloubeidi MA. Impact of EUS in the evaluation of pancreaticobiliary disorders in children. Gastrointest Endosc 2005;62:239-44.
• 19. Delaney L, Applegate KE, Karmazyn B, Akisik MF, Jennings SG. MR cholangiopancreatography in children: feasibility, safety, and initial experience. Pediatr Radiol 2008;38:64-75.
• 20. Tipnis NA, Werlin SL. The use of magnetic resonance cholangiopancreatography in children. Curr Gastroenterol Rep 2007;9:225-9.
• 21. Chavhan GB, Babyn PS, Manson D, Vidarsson L. Pediatric MR cholangiopancreatography: principles, technique, and clinical applications. Radiographics 2008;28:1951-62.
• 22. Manfredi R, Lucidi V, Gui B, Brizi MG, Vecchioli A, Maresca G, Dall'Oglio L, Costamagna G, Marano P. Idiopathic chronic pancreatitis in children: MR cholangiopancreatography after secretin administration. Radiology 2002;224:675-82.
• 23. Manfredi R, Costamagna G, Brizi MG, Maresca G, Vecchioli A, Colagrande C, Marano P. Severe chronic pancreatitis versus suspected pancreatic disease: dynamic MR cholangiopancreatography after secretin stimulation. Radiology 2000;214:849-55.
• 24. Pfau PR, Chelimsky GG, Kinnard MF, Sivak MV, Jr., Wong RC, Isenberg GA, Gurumurthy P, Chak A. Endoscopic retrograde cholangiopancreatography in children and adolescents. J Pediatr Gastroenterol Nutr 2002;35:619-23.
• 25. Issa H, Al-Haddad A, Al-Salem AH. Diagnostic and therapeutic ERCP in the pediatric age group. Pediatr Surg Int 2007;23:111-6.
• 26. Graham KS, Ingram JD, Steinberg SE, Narkewicz MR. ERCP in the management of pediatric pancreatitis. Gastrointest Endosc 1998;47:492-5.
• 27. Kinney TP, Freeman ML. Recent advances and novel methods in pancreatic imaging. Minerva Gastroenterol Dietol 2008;54:85-95.
• 28. Schneider A, Whitcomb DC. Hereditary pancreatitis: a model for inflammatory diseases of the pancreas. Best.Pract.Res.Clin.Gastroenterol. 2002;16:347-363.
• 29. Wilschanski M, Durie PR. Patterns of GI disease in adulthood associated with mutations in the CFTR gene. Gut 2007;56:1153-1163.
• 30. Durno C, Corey M, Zielenski J, Tullis E, Tsui LC, Durie P. Genotype and phenotype correlations in patients with cystic fibrosis and pancreatitis. Gastroenterology 2002;123:1857- 1864.
• 31. Cohn JA, Friedman KJ, Noone PG, Knowles MR, Silverman LM, Jowell PS. Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis. N.Engl.J.Med. 1998;339:653-658.
• 32. Cohn JA, Neoptolemos JP, Feng J, Yan J, Jiang Z, Greenhalf W, McFaul C, Mountford R, Sommer SS. Increased risk of idiopathic chronic pancreatitis in cystic fibrosis carriers. Hum Mutat 2005;26:303-7.
• 33. Cohn JA. Reduced CFTR function and the pathobiology of idiopathic pancreatitis. J.Clin.Gastroenterol. 2005;39:S70-S77.
• 34. Witt H, Luck W, Hennies HC, Classen M, Kage A, Lass U, Landt O, Becker M. Mutations in the gene encoding the serine protease inhibitor, Kazal type 1 are associated with chronic pancreatitis. Nat Genet 2000;25:213-6.
• 35. Pfutzer RH, Barmada MM, Brunskill AP, Finch R, Hart PS, Neoptolemos J, Furey WF, Whitcomb DC. SPINK1/PSTI polymorphisms act as disease modifiers in familial and idiopathic chronic pancreatitis. Gastroenterology 2000;119:615-23.
• 36. Schibli S, Corey M, Gaskin KJ, Ellis L, Durie PR. Towards the ideal quantitative pancreatic function test: analysis of test variables that influence validity. Clin.Gastroenterol.Hepatol. 2006;4:90-97.
• 37. DiMagno EP, Go VL, Summerskill WH. Relations between pancreatic enzyme ouputs and malabsorption in severe pancreatic insufficiency. N.Engl.J Med. 1973;288:813-815.
• 38. Daftary A, Acton J, Heubi J, Amin R. Fecal elastase-1: utility in pancreatic function in cystic fibrosis. J.Cyst.Fibros. 2006;5:71-76.
• 39. Bhardwaj P, Garg PK, Maulik SK, Saraya A, Tandon RK, Acharya SK. A randomized controlled trial of antioxidant supplementation for pain relief in patients with chronic pancreatitis. Gastroenterology. 2009;136:149-159.
• 40. Cremer M, Deviere J, Delhaye M, Baize M, Vandermeeren A. Stenting in severe chronic pancreatitis: results of medium-term follow-up in seventysix patients. Endoscopy 1991;23:171- 6.
• 41. Ponchon T, Bory RM, Hedelius F, Roubein LD, Paliard P, Napoleon B, Chavaillon A. Endoscopic stenting for pain relief in chronic pancreatitis: results of a standardized protocol. Gastrointest Endosc 1995;42:452-6.
• 42. DuBay D, Sandler A, Kimura K, Bishop W, Eimen M, Soper R. The modified Puestow procedure for complicated hereditary pancreatitis in children. J Pediatr Surg 2000;35:343- 8.
• 43. Rollins MD, Meyers RL. Frey procedure for surgical management of chronic pancreatitis in children. J Pediatr Surg 2004;39:817-20.
• 44. Clifton MS, Pelayo JC, Cortes RA, Grethel EJ, Wagner AJ, Lee H, Harrison MR, Farmer DL, Nobuhara KK. Surgical treatment of childhood recurrent pancreatitis. J Pediatr.Surg. 2007;42:1203-1207.
• 45. Andersen DK, Frey CF. The evolution of the surgical treatment of chronic pancreatitis. Ann Surg 2010;251:18- 32.
• 46. Bellin MD, Blondet JJ, Beilman GJ, Dunn TB, Balamurugan AN, Thomas W, Sutherland DE, Moran A. Predicting islet yield in pediatric patients undergoing pancreatectomy and autoislet transplantation for chronic pancreatitis. Pediatr Diabetes 2010;11:227-34.
• 47. Kobayashi T, Manivel JC, Bellin MD, Carlson AM, Moran A, Freeman ML, Hering BJ, Sutherland DE. Correlation of pancreatic histopathologic findings and islet yield in children with chronic pancreatitis undergoing total pancreatectomy and islet autotransplantation. Pancreas 2010;39:57-63.