Gastrointestinal outcomes and confounders in cystic fibrosis

Journal of Pediatric Gastroenterology and Nutrition

Volumn 41, Issue 3, 2005, Pages 273-285

  Borowitz, Drucy ^a   Durie, Peter R ^a   Clarke, Lane L ^a   Werlin, Steven L ^a   Taylor, Christopher J ^a   Semler, John ^a   De Lisle, Robert C ^a   Lewindon, Peter ^a   Lichtman, Steven M ^a   Sinaasappel, Maarten ^a   Baker, Robert D ^a   Baker, Susan S ^a   Verkade, Henkjan J ^a   Lowe, Mark E ^a   Stallings, Virginia A ^a   Janghorbani, Morteza ^a   Butler, Ross ^a   Heubi, James ^a  

^a NONE

Author keywords

[No Author keywords available]

Indexed keywords

PANCREAS ENZYME;

BACTERIAL GROWTH; CONFERENCE PAPER; CYSTIC FIBROSIS; DIET THERAPY; ENZYME THERAPY; GASTROINTESTINAL DISEASE; HUMAN; LIVER DISEASE; MALABSORPTION; MECONIUM ILEUS; PANCREAS INSUFFICIENCY; PATHOGENESIS; PRIORITY JOURNAL; STOMACH EMPTYING;

ANIMALS; CHILD; CYSTIC FIBROSIS; DIETARY FATS; DISEASE MODELS, ANIMAL; EXOCRINE PANCREATIC INSUFFICIENCY; HUMANS; INTESTINAL ABSORPTION; MICE; PANCREATIC ELASTASE; RESPIRATORY TRACT INFECTIONS; TREATMENT OUTCOME;

BACTERIA (MICROORGANISMS);

EID: 24644498948     PISSN: 02772116     EISSN: None     Source Type: Journal    
DOI: 10.1097/01.mpg.0000178439.64675.8d     Document Type: Conference Paper

References (79)

1. FitzSimmons SC, Burkhart GA, Borowitz DS, et al. High-dose pancreatic enzyme supplements and fibrosing colonopathy in children with CF. N Engl J Med 1997;336:1283-9.
2. Federal Register, Vol. 69, No. 82(Docket 2003N-0205), pp. 23410-4. Available at; www.fda.gov/cder/guidance/index.htm.
3. Durie P, Kalnins D, Ellis L. Uses and abuses of enzyme therapy in cystic fibrosis. J Royal Soc Med 1997;91(Suppl 34):2-13.
4. Baker SS, Borowitz D, Duffy L, et al. Pancreatic enzyme therapy and clinical outcomes in patients with cystic fibrosis. J Pediatr 2005;146:189-93.
5. Zielenski J. Genotype and phenotype in cystic fibrosis. Respiration 2000;67:117-33.
6. Couper RTL, Corey M, Moore DJ, et al. Decline of exocrine pancreatic function in cystic fibrosis patients with pancreatic sufficiency. Pediatr Res 1992;32:179-82.
7. Zielinski J, Corey M, Rozmahel R, et al. Detection of a cystic fibrosis modifier locus for meconium ileus on human chromosome 19q13. Nat Genet 1999;22:128-9.
8. Knowles MR, Konstan M, Schluchter M, et al. CF gene modifiers: comparing variation between unrelated individuals with different pulmonary phenotypes. Pediatr Pulmonol 2004;(Suppl 27):139-40.
9. Davidson DJ and Dorin JR. The CF mouse: an important tool for studying cystic fibrosis. Exp Rev Mol Med 2001;12:1-27.
10. Grubb BR, Boucher RC. Pathophysiology of gene-targeted mouse models for cystic fibrosis. Physiol Rev 1999;79:S193-214.
11. Bijvelds MJC, Bronsveld I, Havinga R, et al. Fat absorption in cystic fibrosis mice is impeded by defective lipolysis and post-lipolytic events. Am J Physiol Gastrointest Liver Physiol 2005;288: G646-53.
12. Clarke LL, Harline MC. Dual role of CFTR in cAMP-stimulated HCO3 secretion across murine duodenum. Am J Physiol 1998;274 (4 Pt 1):G718-726.
13. Pratha VS, Hogan DL, Martensson BA, et al. Identification of transport abnormalities in duodenal mucosa and duodenal enterocytes from patients with cystic fibrosis. Gastroenterology 2000;118:1051-60.
14. Clarke LL, Stien X, Walker NM. Intestinal bicarbonate secretion in CF mice. J Pancreas 2001;2(Suppl 4):263-7.
15. + exchange requires CFTR-mediated cell shrinkage of villus epithelium. Gastroenterology 2003;125:1148-63.
16. Rozmahel R, Wilschanski M, Matin A, et al. Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor. Nat Genet 1996;12:280-7.
17. Kent G, Oliver M, Foskett JK, et al. Phenotypic abnormalities in long-term surviving cystic fibrosis mice. Pediatr Res 1996;40:233-41.
18. Durie PR, Kent G, Phillips MJ, Ackerly CA. Characteristic multiorgan pathology of CF in a long-living cystic fibrosis transmembrane regulator knockout murine model. Am J Pathol 2004;164:1481-93.
19. Norkina O, Kaur S, Ziemer D, De Lisle RC. Inflammation of the cystic fibrosis mouse small intestine. Am J Physiol Gastrointest Liver Physiol 2004;286:G1032-41.
20. Clarke LL, Gawenis LR, Bradford EM, et al. Abnormal Paneth cell granule dissolution and compromised resistance to bacterial colonization in the intestine of CF mice. Am J Physiol Gastrointest Liver Physiol 2004;286:G1050-8.
21. Gallagher AM, Gottlieb RA. Proliferation, not apoptosis, alters epithelial cell migration in small intestine of CFTR null mice. Am J Physiol 2001;281:G681-7.
22. Freedman SD, Katz MH, Parker EM, et al. A membrane-lipid imbalance plays a role in the phenotypic expression of CF in cftr -/- mice. Proc Natl Acad Sci USA 1999;96:13995-4000.
23. Werner A, Bongers ME, Bijvelds MJ et al. No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis. J Lipid Res 2004;45:2277-86.
24. Hardcastle J, Harwood MD, Taylor CJ. Small intestinal glucose absorption in cystic fibrosis: a study in human and transgenic ΔF508 cystic fibrosis mouse tissues. J Pharm Pharmacol 2004;56:329-38.
25. +-glucose cotransport in intestinal epithelia. Am J Physiol 2001;281:C1533-41.
26. Ganapathy V, Ganapathy ME, Leibach FH. Intestinal transport of peptides and amino acids. Jn: Barrett KE and Donowitz M, eds. Gastrointestinal Transport Molecular Physiology (Current Topics in Membranes, Vol. 50). San Diego: Academic Press 2001:379-412.
27. Gawenis LR, Hut H, Bot AGM, et al. Electroneutral sodium absorption and electrogenic anion secretion across murine small intestine are regulated in parallel. Am J Physiol 2004;287:G1140-9.
28. Roulet M, Weber AM, Paradis Y, et al. Gastric emptying and lingual lipase activity in cystic fibrosis. Pediatr Res 1980;14:1360-2.
29. Cavell B. Gastric emptying in infants with cystic fibrosis. Acta Paediatr Scand 1981;70:635-8.
30. Smith HL, Hollins GW, et al. Gastric emptying of liquids in cystic fibrosis. Acta Universitatis Carolinae - Medica 1990;36:161-4.
31. Carney BI, Jones KL, et al. Gastric emptying of oil and aqueous meal components in pancreatic insufficiency: effects of posture and on appetite. Am J Physiol 1995;268(6 Pt 1):G925-32.
32. Cucchiara S, Raia V, Horowitz M, et al. Ultrasound measurement of gastric emptying time in patients with cystic fibrosis and effect of ranitidine on delayed gastric emptying. Pediatr 1996;128:485-8.
33. Collins CE, Francis JL, Thomas P, et al. Gastric emptying time is faster in cystic fibrosis. J Pediatr Gastroenterol Nutr 1997;25:492-8.
34. Symonds E, Omari T, Webster JM, et al. Relation between pancreatic lipase activity and gastric emptying rate in children with cystic fibrosis. J Pediatr 2003;143:772-5.
35. Armand M, Hamosh M, Philpott JR, et al. Gastric function in children with cystic fibrosis: effect of diet on gastric lipase levels and fat digestion. Pediatr Res 2004;55:457-65.
36. Taylor CJ, Hillel PG, Ghosal S, et al. Gastric emptying and intestinal transit of pancreatic enzyme supplements in cystic fibrosis. Arch Dis Child 1999;80:149-52.
37. Dominguez-Munoz JE, Iglesias-Garcia J, Iglesias-Rey M, Figueiras A, Vilarino-Insua M. Effect of the administration schedule on the therapeutic efficacy of oral pancreatic enzyme supplements in patients with exocrine pancreatic insufficiency: a randomized, three-way crossover study. Aliment Pharmacol Ther 2005;21:993-1000.
38. DiMagno EP. Gastric acid suppression and treatment of severe exocrine pancreatic insufficiency. Best Pract Res Clin Gastroenterol 2001;15:477-86.
39. Proesmans M, De Boeck K. Omeprazole, a proton pump inhibitor, improves residual steatorrhoea in cystic fibrosis patients treated with high dose pancreatic enzymes. Eur J Pediatr 2003;162:760-3.
40. Tso P. Intestinal lipid absorption. In: Johnson LR, ed. Physiology of the Gastrointestinal Tract. New York: Raven Press, 1994:1867-1907.
41. Lowe ME. The triglyceride lipases of the pancreas. J Lipid Res 2002;43:2007-16.
42. Kalivianakis M, Minich DM, Bijleveld CMA, et al. Fat malabsorption in cystic fibrosis patients receiving enzyme replacement therapy is due to impaired intestinal uptake of long-chain fatty acids. Am J Clin Nutr 1999;69:127-34.
43. Innis SM, Davidson GF, Chen A, et al. Increased plasma homocysteine and s-adenosylhomocysteine and decreased methionine is associated with altered phosphatidylcholine and phosphatidylethanolamine in cystic fibrosis. J Pediatr 2003;143:351-6.
44. O'Brien S, Mulcahy H, Fenlon H, et al. Intestinal bile acid malabsorption in cystic fibrosis. Gut 1993;34:1137-41.
45. Van Biervliet S, Eggermont E, Marien P, Hoffman I, Veereman G. Combined impact of mucosal damage and of cystic fibrosis on the small intestinal brush border enzyme activities. Acta Clin Belg 2003;58:220-4.
46. Pelton NS, Tivey DR, Howarth GS, Davidson GP, Butler RN. A novel breath test for the non-invasive assessment of small intestinal mucosal injury following methotrexate administration in the rat. Scand J Gastroenterol 2004;39:1015-6.
47. Singh VV, Toskes PP. Small bowel bacterial overgrowth: presentation, diagnosis, and treatment. Curr Gastroenterol Rep 2003;5:365-72.
48. Lewindon PJ, Robb TA, Moore DJ, Davidson GP, Martin AJ. Bowel dysfunction in cystic fibrosis: importance of breath testing. J Paediatr Child Health 1998;34:79-82.
49. Fridge J, Castillo R, Conrad C, Gerson L, Cox K. Small bowel bacterial overgrowth in cystic fibrosis. Gastroenterol 2005;128 (Suppl 2):S1025.
50. Romagnuolo J, Schiller D, Bailey RJ. Using breath tests wisely in a gastroenterology practice. Am J Gastroenterol 2002;97:1113-26.
51. Lichtman S. Bacterial overgrowth. In: Walker WA, Durie PR, Hamilton JR, Walker-Smith JA, Watkins JB, eds. Pediatric Gastrointestinal Disease, 4th ed. Philadelphia: BC Decker Inc.; 2004:569-82.
52. Riordan SM, McIver CJ, Walker BM, et al. The lactulose breath hydrogen test of SBBO. Am J Gastroenterol 1996;91:1795-803.
53. 13C-breath tests to measure fat digestion. Br J Nutrit 1998;79:541-5.
54. Norkina O, Burnett TG, De Lisle RC. Bacterial overgrowth in the cystic fibrosis transmembrane conductance regulator null mouse small intestine. Infect Immun 2004;72:6040-9.
55. Roberts DM, Craft JC, Mather FJ, Davis SH, Wright JA. Prevalence of giardiasis in patients with cystic fibrosis. J Pediatr 1988;112:555-9.
56. Welkon CJ, Long SL, Thompson M, Gilligan PH. Clostridium difficile in patients with cystic fibrosis. Am J Dis Child 1985;139:805-8.
57. Wu TC, McCarthy VP, Gill VJ. Isolate rate and toxigenic potential of clostridium difficile isolates from patients with cystic fibrosis. J Infect Dis 1983;148:176.
58. Lloyd-Still J. Crohn's disease and cystic fibrosis. Dig Dis Sci 1994;39:880-5.
59. Hill ID, Dirks MH, Liptak GS, et al. Guidelines for the diagnosis and treatment of celiac disease in children: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 2004;40:1-19.
60. Elson CO, Ballew M, Barnard JA, et al. National Institutes of Health Consensus Development Conference Statement: Celiac Disease. June 28-30, 2004. Available at: http://consensus.nih. gov/cons/118/118cdc_intro.htm.
61. Farrell RJ, Kelly CP. Diagnosis of celiac sprue. Am J Gastroenterol 2001;96:3237-46.
62. Fasano A, Berti I, Gerarduzzi T, et al. Prevalence of celiac disease in at-risk and not-at-risk groups in the United States: a large multicenter study. Arch Intern Med 2003;163:286-92.
63. MacDonald TT, Monteleone G. Immunity, inflammation, and allergy in the gut. Science 2005;307:1920-25.
64. Ruemelle FM, Targan SR, Levy G, et al. Diagnostic accuracy of serological assays in pediatric inflammatory bowel disease. Gastroenterol 1998;115:822-29.
65. Borowitz D, Goss CH, Stevens C, et al. Safety and preliminary clinical activity of a novel pancreatic enzyme preparation, TheraCLEC-Total, containing pancrease, lipase and amylase for the treatment of exocrine pancreatic insufficiency. Pediatr Pulmonol 2003;Suppl 25:339.
66. Stern RC, Eisenberg JD, Wagener JS, et al. A comparison of the efficacy and tolerance of pancrelipase and placebo in the treatment of steatorrhea in cystic fibrosis patients with clinical pancreatic insufficiency. Am J Gastroenterol 2000;95:1932-8.
67. Konstan MW, Stern RC, Trout JR, et al. Ultrase MT12 and Ultrase MT20 in the treatment of exocrine pancreatic insufficiency in cystic fibrosis: safety and efficacy. Aliment Pharmacol Ther 2004;20:1365-71.
68. Goldstein R, Blondheim O, Levy E, Stankiewicz H, Freier S. The fatty meal test: an alternative to stool fat analysis. Am J Clin Nutr 1983;38:763-8.
69. Stallings VA, Schall JI, Mascarenhas M. Malabsorption blood test (MBT): a novel approach to quantify steatorrhea. Pediatr Pulmonol 2003;(Suppl 25):338.
70. Davidson GP, Butler RN. Breath tests in pediatric gastroenterology. In: Walker A, Durie P, Hamilton J, Walker-Smith J, eds. Pediatric Gastrointestinal Disease. 3rd ed. Philadelphia: BC Deker, Inc. 2000:1529-37.
71. Ghoos YF, Maes BD, Geypens BJ, et al. Measurement of gastric emptying rate of solid by means of a carbon-labeled octanoic acid breath test. Gastroenterology 1993;104:1640-47.
72. Ghoos YF, Vantrappen GR, Rutgeerts PJ, Schurmans PC. A mixed-triglyceride breath test for intraluminal fat digestive activity. Digestion 1981;22:239-47.
73. 13C-triolein breath test for fat malabsorption in adult patients with cystic fibrosis. J Gastroenterol Hepatol 2004;19:448-53.
74. Kalivianakis M, Elstrodt J, Havinga R, et al. Validation in an animal model of the carbon 13-labeled mixed triglyceride breath test for the detection of intestinal fat malabsorption. J Pediatr 1999;135:444-50.
75. Schuette SA, Janghorbani M, Young VR, Weaver CM. Dysprosium as a nonabsorbable marker for studies of mineral absorption with stable isotopes tracers in human subjects. J Am Coll Nutr 1993;12:307-15.
76. Schuette SA, Janghorbani M, Cohen MB, et al. Dysprosium chloride as a nonabsorbable gastrointestinal marker for studies of stable-isotope-labeled triglyceride excretion in man. J Am Coll Nutr 2003;22:379-87.
77. 13C-labeled triglycerides. J Am Coll Nutr 2003;22:511-18.
78. Metcalfe LD, Schmitz AA, Pelka JR. Rapid preparation of fatty acid esters from lipids for gas chromatographic analysis. Anal Chem 1966;38:514-5.
79. Jandacek RJ, Heubi JE, Tso P. A novel, noninvasive method for measurement of intestinal fat absorption. Gastroenterology 2004;127:139-44.