Current Gastroenterology Reports

, Volume 7, Issue 3, pp 227–233 | Cite as

Pancreatic exocrine function in patients with cystic fibrosis

  • Susan S. Baker
  • Drucy Borowitz
  • Robert D. Baker
Article

Abstract

Pancreatic insufficiency in cystic fibrosis (CF) is associated with more severe disease and requires replacement therapy. Outcome measures such as growth and number of stools, frequency of abdominal pain, and flatulence have often been used to identify pancreatic-insufficient patients and to adjust the dose of replacement enzymes. Unfortunately, some patients with CF are misclassified, and approximately 9% do not receive therapy appropriate for their pancreatic exocrine functional status. Growth, number of stools, frequency of abdominal pain, and flatulence cannot be used to adjust enzyme doses.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References and Recommended Reading

  1. 1.
    OMIM (Online Mendelian Inheritance in Man). http:// www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=602421 Accessed 12/14/04.Google Scholar
  2. 2.
    Choi JY, Muallem D, Kiselyov K, et al.: Aberrant CFTR-dependent HCO(-3) transport in mutations associated with cystic fibrosis. Nature 2001, 410:94–97.PubMedCrossRefGoogle Scholar
  3. 3.
    Reddy MM, Light MJ, Quinton PM: Activation of the epithelial Na(+) channel (ENaC) requires CFTR CI(-) channel function. Nature 1999, 402:301–304. Identified the product of the gene that is at fault in cystic fibrosis, cystic fibrosis transmembrane conductance regulator (CFTR), and its function as a small-conductance chloride channel activated by phosphorylation.PubMedCrossRefGoogle Scholar
  4. 4.
    Tucker JA, Spock A, Spicer SS, et al.: Inspissation of pancreatic zymogen material in cystic fibrosis. Ultrastruct Pathol 2003, 27:323–335.PubMedGoogle Scholar
  5. 5.
    Guy-Crotte O, Carrere J, Figarella C: Exocrine pancreatic function in cystic fibrosis. Eur J Gastroenterol Hepatol 1996, 8:755–759.PubMedGoogle Scholar
  6. 6.
    Guimbaud R, Moreau JA, Bouisson M, et al.: Intraduodenal free fatty acids rather than triglycerides are responsible for the release of CCK in humans. Pancreas 1997, 14:76–82.PubMedCrossRefGoogle Scholar
  7. 7.
    DiMagno EP, Go VLW, Summerskill WHJ: Relations between pancreatic enzyme outputs and malabsorption in severe pancreatic insufficiency. N Engl J Med 1973, 288:813–815. Described clinical and functional assessment of pancreatic insufficiency.PubMedCrossRefGoogle Scholar
  8. 8.
    Keller J, Runzi M, Goebell H, Layer P: Duodenal and ileal nutrient deliveries regulate human intestinal motor and pancreatic responses to a meal. Am J Physiol 1988, 272:G632-G637. Demonstrated altered motility in exocrine pancreatic insufficiency.Google Scholar
  9. 9.
    Layer P, von der Ohe MR, Holst JJ, et al.: Altered postprandial motility in chronic pancreatitis: role of malabsorption. Gastroenterology 1997, 112:1624–1634.PubMedCrossRefGoogle Scholar
  10. 10.
    Layer P, Keller J, et al.: Pancreatic enzymes: secretion and luminal nutrient digestion in health and disease. J Clin Gastroenterol 1999, 28:3–10.PubMedCrossRefGoogle Scholar
  11. 11.
    Zielinski J: Genotype and phenotype in cystic fibrosis. Respir 2000, 67:117–133.CrossRefGoogle Scholar
  12. 12.
    Kerem E, Corey M, Kerem B, et al.: The relationship between genotype and phenotype in cystic fibrosis - analysis of the most common mutation (delta F508). N Engl J Med 1990, 323:1517–1522.PubMedCrossRefGoogle Scholar
  13. 13.
    Durno C, Corey M, Zielenski J, et al.: Genotype and phenotype correlations in patients with cystic fibrosis and pancreatitis. Gastroenterology 2002, 123:1857–1864.PubMedCrossRefGoogle Scholar
  14. 14.
    Cystic Fibrosis Foundation: Patient Registry: 1998 Annual Data Report to the Center Directors. Bethesda, MD: CFF; 1998.Google Scholar
  15. 15.
    Cooper R: Pancreatic function tests. In Pediatric Gastrointestinal Disease. Edited by Walker, et al. Baltimore, MD: Mosby; 1996:1621–1634.Google Scholar
  16. 16.
    Cystic Fibrosis Foundation: Patient Registry: 2003 Annual Data Report to the Center Directors. Bethesda, MD: CFF; 2003.Google Scholar
  17. 17.
    Durie PR, Forstner GG, Gaskin KJ, et al.: Age-related alterations of immunoreactive pancreatic cationic trypsinogen in sera from cystic fibrosis patients with and without pancreatic insufficiency. Pediatr Res 1986, 20:209–213.PubMedCrossRefGoogle Scholar
  18. 18.
    Soldan W, Henker J, Sprossig C: Sensitivity and specificity of quantitative determination of pancreatic elastase-1 in feces of children. J Pediatr Gastroenterol Nutr 1997, 24:53–55.PubMedCrossRefGoogle Scholar
  19. 19.
    Beharry S, Ellis L, Corey M, et al.: How useful is fecal pancreatic elastase 1 as a marker of exocrine pancreatic disease? J Pediatr 2002, 141:84–90.PubMedCrossRefGoogle Scholar
  20. 20.
    Loser C, Molgaard A, Folsch UR: Faecal elastase-1: a novel, highly sensitive, and specific tubeless pancreatic function test. Gut 1996, 39:580–586.PubMedGoogle Scholar
  21. 21.
    Nissler K, Katte IV, Huebner A, Henker J: Pancreatic elastase-1 in feces of preterm and term infants. J Pediatr Gastroenterol Nutr 2001, 33:28–31.PubMedCrossRefGoogle Scholar
  22. 22.
    Stein J, Jung M, Sziegoleit A, et al.: Immunoreactive elastase-1: clinical evaluation of a new noninvasive test of pancreatic function. Clin Chem 1996, 42:222–226.PubMedGoogle Scholar
  23. 23.
    Borowitz D, Baker RD, Stallings V: Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002, 35:246–259. Reviews nutrition in CF.PubMedCrossRefGoogle Scholar
  24. 24.
    Kraemer R, Rudeberg A, Hadom B, et al.: Relative underweight in cystic fibrosis and its prognostic value. Acta Paediatr Scand 1978, 67:33–37.PubMedGoogle Scholar
  25. 25.
    Corey M, McLaughlin FJ, Williams M, et al.: A comparison of survival, growth and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol 1988, 41:588–591.CrossRefGoogle Scholar
  26. 26.
    Kerem E, Reisman J, Corey M, et al.: Prediction of mortality in patients with cystic fibrosis. N Engl J Med 1992, 326:1187–1191.PubMedCrossRefGoogle Scholar
  27. 27.
    Dalzell AM, Shepherd RW, Dean SB, et al.: Nutritional rehabilitation in cystic fibrosis: a 5 year follow-up study. J Pediatr Gastroenterol Nutr 1992, 15:141–145.PubMedCrossRefGoogle Scholar
  28. 28.
    Pencharz PB, Durie PR: Nutritional management of cystic fibrosis. Ann Rev Nutr 1993, 13:111–136.CrossRefGoogle Scholar
  29. 29.
    Beker LT, Russek-Cohen E, Fink RJ: Stature as a prognostic factor in cystic fibrosis survival. J Am Diet Assoc 2001, 101:438–442.PubMedCrossRefGoogle Scholar
  30. 30.
    US Food and Drug Administration Center for Drug Evaluation and Research. http://www.fda.gov/cder/drug/infopage/ pancreatic_drugs/pancreatic_QA.htm Accessed 12/24/04Google Scholar
  31. 31.
    Borowitz D, Baker SS, Duffy L, et al.: Use of fecal elastase-1 to classify pancreatic status in patients with cystic fibrosis [see comment]. J Pediatr 2004, 145:322–326. Demonstrates misclassification of CF patients with respect to exocrine pancreatic function.PubMedCrossRefGoogle Scholar
  32. 32.
    Farrell PM, Kosorok MR, Rock MJ, et al.: Disease in patients with cystic fibrosis diagnosed through neonatal screening or after delays associated with traditional diagnosis. Pediatr Pulmonol 2002, (Suppl 24):319.Google Scholar
  33. 33.
    Walkowiak J, Witmanowski H, Strzykala K, et al.: Inhibition of endogenous pancreatic enzyme secretion by oral pancreatic enzyme treatment. Eur J Clin Invest 2003, 33:65–69.PubMedCrossRefGoogle Scholar
  34. 34.
    Gaskin K, Gurwitz D, Durie P, et al.: Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption. J Pediatr 1982, 100:857–862.PubMedCrossRefGoogle Scholar
  35. 35.
    Kerem E, Corey M, Kerem B, et al.: The relationship between genotype and phenotype in cystic fibrosis: analysis of the most common mutation ([DELTA]F508). N Engl J Med 1990, 323:1517–1522.PubMedCrossRefGoogle Scholar
  36. 36.
    Zielinski J: Genotype and phenotype in cystic fibrosis. Respiration 2000, 67:117–133.CrossRefGoogle Scholar
  37. 37.
    Walkowiak J, Herzig KH, Witt M, et al.: Analysis of exocrine pancreatic function in cystic fibrosis: one mild CFTR mutation does not exclude pancreatic insufficiency. Eur J Clin Invest 2001, 31:796–801.PubMedCrossRefGoogle Scholar
  38. 38.
    Haynes RB, McDonald HP, Garg AX: Helping patients follow prescribed treatment: clinical applications. JAMA 2002, 288:2880–2883.PubMedCrossRefGoogle Scholar
  39. 39.
    Stephenson BJ, Rowe BH, Haynes RB, et al.: Is this patient taking the treatment as prescribed? JAMA 1993, 269:2779–2781.PubMedCrossRefGoogle Scholar
  40. 40.
    Borowitz DS, Grand RJ, Durie PR: Consensus Committee: Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. J Pediatr 1995, 127:681–684.PubMedCrossRefGoogle Scholar
  41. 41.
    CF and Nutrition. http://www.cff.org Accessed 3/31/05.Google Scholar
  42. 42.
    Baker SS, Borowitz D, Duffy L, et al.: Pancreatic enzyme therapy and clinical outcomes in patients with cystic fibrosis. J Pediatr 2005, 146:189–193. Demonstrates lack of relationship between pancreatic enzyme replacement and clinical symptoms.PubMedCrossRefGoogle Scholar
  43. 43.
    National Center for Health Statistics. http://www.cdc.gov/ nchs/products/pubs/pubd/hestats/obese/obse99figs1and2.htmGoogle Scholar
  44. 44.
    Dray X, Marteau P, Bienvenu T, et al.: Discussion on genotype and phenotype correlations in patients with cystic fibrosis and pancreatitis. Gastroenterology 2003, 125:1286.PubMedCrossRefGoogle Scholar
  45. 45.
    Modolell I, Alverez A, Guarner L, et al.: Gastrointestinal, liver and pancreatic involvement in adult patients with cystic fibrosis. Pancreas 2001, 22:395–399.PubMedCrossRefGoogle Scholar
  46. 46.
    Moreno GE, Ibanez AJ, Rico SP, et al.: Recurrent acute pancreatitis as a complication of cystic fibrosis: report of one case treated surgically. Annali Italiani di Chirurgia 1991, 62:345–347.Google Scholar
  47. 47.
    Del Rosario JF, Putnam PE, Orenstein DM: Chronic pancreatitis in a patient with cystic fibrosis and clinical pancreatic insufficiency. J Pediatr 1995, 126:951–952.CrossRefGoogle Scholar
  48. 48.
    Apley J, Naish N: Recurrent abdominal pain: a field survey of 1,000 school children. Arch Dis Child 1958, 33:165–170.PubMedCrossRefGoogle Scholar
  49. 49.
    Hyams JS, Burke G, Davis PM, et al.: Abdominal pain and irritable bowel syndrome in adolescents: a community-based study. J Pediatr 1966, 129:220–226.Google Scholar
  50. 50.
    Rasquin-Weber A, Hyman PE, Cucchiara S, et al.: Childhood functional gastrointestinal disorders. Gut 1000, 45(suppl II): II60-II68.Google Scholar
  51. 51.
    Stewart WF, Liberman JN, Sandler RS, et al.: Epidemiology of constipation (EPOC) study in the United States: relation of clinical subtypes to sociodemographic features. Am J Gastroenterol 1999, 94:3530–3540.PubMedCrossRefGoogle Scholar
  52. 52.
    Loening-Baucke V: Constipation in children. N Engl J Med 1998, 339:1155–1156.PubMedCrossRefGoogle Scholar
  53. 53.
    Rubinstein S, Moss R, Lewiston N: Constipation and meconium ileus equivalent in patients with cystic fibrosis. Pediatrics 1986, 78:473–479.PubMedGoogle Scholar
  54. 54.
    Baker SS, Liptak GS, Colletti RB, et al.: Constipation in infants and children: Evaluation and treatment. J Pediatr Gastroenterol Nutr 1999, 29:612–626.PubMedCrossRefGoogle Scholar
  55. 55.
    Schmitz J: Maldigestion and malabsorption. In Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, Management, edn 2. Edited by Walker WA, Durie PR, Hamilton JR, et al. St. Louis, MO: Mosby; 2000:51–58.Google Scholar

Copyright information

© Current Science Inc. 2005

Authors and Affiliations

  • Susan S. Baker
    • 1
  • Drucy Borowitz
  • Robert D. Baker
  1. 1.Digestive Diseases and Nutrition CenterWomen and Children’s HospitalBuffaloUSA

Personalised recommendations