Gastrointestinal complications of cystic fibrosis

Cystic Fibrosis

Volumn , Issue , 2016, Pages 266-284

  Mascarenhas, Maria ^a   Maqbool, Asim ^a  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 84879884268     PISSN: None     EISSN: None     Source Type: Book    
DOI: None     Document Type: Chapter

References (136)

1. Borowitz D, Durie PR, Clarke LL, et al. Gastrointestinal outcomes and confounders in cystic fibrosis. J Pediatr Gastroenterol Nutr 2005; 41(3):273-285.
2. Rogers CS, Stoltz Da, Meyerholz DK, et al. Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. Science 2008; 321(5897):1837-1841.
3. Durie PR, Forstner GG. Pathophysiology of the exocrine pancreas in cystic fibrosis. J R Soc Med 1989; 82(suppl. 16):2-10.
4. Schäppi MG, Roulet M, Rochat T, et al. Electrogastrography reveals post-prandial gastric dysmotility in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2004; 39(3): 253-256.
5. Robinson PJ, Smith AL, Sly PD. Duodenal pH in cystic fibrosis and its relationship to fat malabsorption. Dig Dis Sci 1990; 35(10):1299-12304.
6. Barraclough M, Taylor CJ. Twenty-four hour ambulatory gastric and duodenal pH profiles in cystic fibrosis: effect of duodenal hyperacidity on pancreatic enzyme function and fat absorption. J Pediatr Gastroenterol Nutr 1996; 23(1):45-50.
7. Nouri-Sorkhabi MH, Chapman BE, Kuchel PW, et al. Parallel secretion of pancreatic phospholipase A(2), phospholipase A(1), lipase, and colipase in children with exocrine pancreatic dysfunction. Pediatr Res 2000; 48(6):735-740.
8. Chen AH, Innis SM, Davidson AG, et al. Phosphatidylcholine and lysophosphatidylcholine excretion is increased in children with cystic fibrosis and is associated with plasma homocysteine, S-adenosylhomocysteine, and S-adenosylmethionine. Am J Clin Nutr 2005; 81(3):686-691.
9. Barrett KE, Keely SJ. Chloride secretion by the intestinal epithelium: molecular basis and regulatory aspects. Annu Rev Physiol 2000; 62:535-572.
10. Dalzell AM, Freestone NS, Billington D, et al. Small intestinal permeability and oro-caecal transit time in cystic fibrosis. Arch Dis Child 1990; 65:585-588.
11. 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-G1058.
12. Fridge J, Castillo R, Conrad C, et al. Small bowel bacterial overgrowth in cystic fibrosis. Gastroenterology 2005; 128(suppl. 2):S1025.
13. De Lisle RC. Altered transit and bacterial overgrowth in the cystic fibrosis mouse small intestine. Am J Physiol Gastrointest Liver Physiol 2007; 293(1):G104-G111.
14. Norkina O, Kaur S, Ziemer D, et al. Inflammation of the cystic fibrosis mouse small intestine. Am J Physiol Gastrointest Liver Physiol 2004; 286:G1032-G1041.
15. O'Brien S, Mulcahy H, Fenlon H, et al. Intestinal bile acid malabsorption in cystic fibrosis. Gut 1993; 34:1137-1141.
16. Guéant JL, Champigneulle B, Gaucher P, et al. Malabsorption of vitamin B12 in pancreatic insufficiency of the adult and of the child. Pancreas 1990; 5(5):559-567.
17. 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; 69:127-134.
18. Murphy J, Laiho K, Wootton S. Fat malabsorption in cystic fibrosis patients. Am J Clin Nutr 1999; 69(1):127-134.
19. De Lisle RC, Meldi L, Flynn M, et al. Altered eicosanoid metabolism in the cystic fibrosis mouse small intestine. J Pediatr Gastroenterol Nutr 2008; 47(4):406-416.
20. Ng SM, Jones AP. Drug therapies for reducing gastric acidity in people with cystic fibrosis. Cochrane Database Syst Rev 2003; (2):CD003424.
21. Francisco MP, Wagner MH, Sherman JM, et al. Ranitidine and omeprazole as adjuvant therapy to pancrelipase to improve fat absorption in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002; 35(1):79-83.
22. De Lisle RC, Roach E, Jansson K. Effects of laxative and N-acetylcysteine on mucus accumulation, bacterial load, transit, and inflammation in the cystic fibrosis mouse small intestine. Am J Physiol Gastrointest Liver Physiol 2007; 293(3):G577-G584.
23. Kopelman H, Durie P, Gaskin K, et al. Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. N Engl J Med 1985; 312(6):329-334.
24. Scheele GA, Fukuoka SI, Kern HF, et al. Pancreatic dysfunction in cystic fibrosis occurs as a result of impairments in luminal pH, apical trafficking of zymogen granule membranes, and solubilization of secretory enzymes. Pancreas 1996; 12(1):1-9.
25. Waters DL, Dorney SF, Gaskin KJ, et al. Pancreatic function in infants identified as having cystic fibrosis in a neonatal screening program. N Engl J Med 1990; 322(5):303-308.
26. Walkowiak J, Sands D, Nowakowska A, et al. Early decline of pancreatic function in cystic fibrosis patients with class 1 or 2 CFTR mutations. J Pediatr Gastroenterol Nutr 2005; 40(2):199-201.
27. Walkowiak J, Nousia-Arvanitakis S, Henker J, et al. Indirect pancreatic function tests in children. J Pediatr Gastroenterol Nutr 2005; 40(2):107-114.
28. Gaskin K, Waters D, Dorney S, et al. Assessment of pancreatic function in screened infants with cystic fibrosis. Pediatr Pulmonol Suppl 1991; 7:69-71.
29. Durno C, Corey M, Zielenski J, et al. Genotype and phenotype correlations in patients with cystic fibrosis and pancreatitis. Gastroenterology 2002; 123(6):1857-1864.
30. Ahmed N, Corey M, Forstner G, et al. Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas. Gut 2003; 52(8): 1159-1164.
31. FitzSimmons SC. Cystic Fibrosis Foundation Patient Registry 1995; Annual Data Report. Bethesda, MD: Cystic Fibrosis Foundation, 1996.
32. Walkowiak J, Lisowska A, Przyslawski J, et al. Faecal elastase-1 test is superior to faecal lipase test in the assessment of exocrine pancreatic function in cystic fibrosis. Acta Paediatr 2004; 93(8):1042-1045.
33. Walkowiak J, Herzig KH, Strzykala K, et al. Fecal elastase-1 is superior to fecal chymotrypsin in the assessment of pancreatic involvement in cystic fibrosis. Pediatrics 2002; 110(1 pt 1):e7.
34. 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(1):84-90.
35. Schneider A, Funk B, Caspary W, et al. Monoclonal versus polyclonal ELISA for assessment of fecal elastase concentration: pitfalls of a new assay. Clin Chem 2005; 51(6): 1052-1054.
36. Borowitz D, Lin R, Baker SS. Comparison of monoclonal and polyclonal ELISAs for fecal elastase in patients with cystic fibrosis and pancreatic insufficiency. J Pediatr Gastroenterol Nutr 2007; 44(2):219-223.
37. Walkowiak J, Nousia-Arvanitakis S, Cade A, et al. Fecal elastase-1 cut-offlevels in the assessment of exocrine pancreatic function in cystic fibrosis. J Cyst Fibros 2002; 1(4):260-264.
38. Borowitz D, Baker SS, Duffy L, et al. Use of fecal elastase-1 to classify pancreatic status in patients with cystic fibrosis. J Pediatr 2004; 145(3):322-326.
39. Walkowiak J, Lisowska A. Re: fecal elastase: pancreatic status verification and influence on nutritional status in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2006; 42(1):117.
40. Cohen JR, Schall JI, Ittenbach RF, et al. Fecal elastase: pancreatic status verification and influence on nutritional status in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2005; 40(4):438-444.
41. Walkowiak J, Cichy WK, Herzig KH. Comparison of fecal elastase-1 determination with the secretin-cholecystokinin test in patients with cystic fibrosis. Scand J Gastroenterol 1999; 34(2):202-207.
42. Weintraub A, Blau H, MussaffiH, et al. Exocrine pancreatic function testing in patients with cystic fibrosis and pancreatic sufficiency: a correlation study. J Pediatr Gastroenterol Nutr 2009; 48(3):306-310.
43. Walkowiak J, Nousia-Arvanitakis S, Agguridaki C, et al. Longitudinal follow-up of exocrine pancreatic function in pancreatic sufficient cystic fibrosis patients using the fecal elastase-1 test. J Pediatr Gastroenterol Nutr 2003; 36(4):474-478.
44. Littlewood JM, Wolfe SP, Conway SP. Diagnosis and treatment of intestinal malabsorption in cystic fibrosis. Pediatr Pulmonol 2006; 41(1):35-49.
45. Schibli S, Durie PR, Tullis ED. Proper usage of pancreatic enzymes. Curr Opin Pulm Med 2002; 8(6):542-546.
46. Borowitz DS, Grand RJ, Durie PR. Use of pancreatic enzyme supplements for patients with CF in the context of fibrosing colonopathy. Consensus Committee. J Pediatr 1995; 127(5): 681-684.
47. Fridell JA, Vianna R, Kwo PY, et al. Simultaneous liver and pancreas transplantation in patients with cystic fibrosis. Transplant Proc 2005; 37(8):3567-3569.
48. Young AL, Peters CJ, Toogood GJ, et al. A combined liver-pancreas en-bloc transplant in a patient with cystic fibrosis. Transplantation 2005; 80(5):605-607.
49. Fridell JA, Wozniak TC, Reynolds JM, et al. Bilateral sequential lung and simultaneous pancreas transplant: a new approach for the recipient with cystic fibrosis. J Cyst Fibros 2008; 7(4):280-284.
50. Cohn JA, Friedman KJ, Noone PG, et al. Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis. N Engl J Med 1998; 339(10):653-658.
51. Keiles S, Kammesheidt A. Identification of CFTR, PRSS1, and SPINK1 mutations in 381 patients with pancreatitis. Pancreas 2006; 33(3):221-227.
52. Blackman SM, Deering-Brose R, McWilliams R, et al. Relative contribution of genetic and nongenetic modifiers to intestinal obstruction in cystic fibrosis. Gastroenterology 2006; 131(4):1030-1039.
53. Del Pin CA, Czyrko C, Zeigler MM, et al. Management and survival of meconium ileus-a 30-year review. Ann Surg 1992; 215:179-185.
54. Jawaheer J, Khalil B, Plummer T, et al. Primary resection and anastamosis for complicated meconium ileus: a safe procedure? Pediatr Surg Intl 2007; 23(11):1091-1093.
55. Mushtaq I, Wright VM, Drake DP, et al. Meconium ileus secondary to cystic fibrosis. The East London experience. Pediatr Surg Int 1998; 13(5-6):365-369.
56. Farrell PM, Lai HZ, Li Z. Evidence on improved outcomes with early diagnosis of cystic fibrosis through neonatal screening: enough is enough. J Pediatr 2005; 147(3):s30-s36.
57. Munck A, Gerardin M, Alberti C, et al. Clinical outcome of cystic fibrosis presenting with or without meconium ileus: a matched cohort study. J Pediatr Surg 2006; 41(9):1556-1560.
58. Li Z, Lai HJ, Kosorok MR, et al. Longitudinal pulmonary status of cystic fibrosis children with meconium ileus. Pediatr Pulmonol 2004; 38(4):277-284.
59. Mascarenhas MR. Treatment of gastrointestinal problems in cystic fibrosis. Curr Treat Options Gastroenterol 2003; 6:427-441.
60. Millar-Jones L, Goodchild MC. Cystic fibrosis, pancreatic sufficiency and distal intestinal obstruction syndrome: a report of 4 cases. Acta Pediatr 1995; 84:577-578.
61. Zielenski J, Corey M, Rozmahel R, et al. Detection of a cystic fibrosis modifier locus for meconuim ileus on human chromosome 19q13. Nat Genet 1999, 2:128-129.
62. Minkes RK, Langer JC, Skinner MA, et al. Intestinal obstruction after lung transplantation in children with CF. J Pediatr Surg 1999; 34:1489-1493.
63. Morton JR, Ansari N, Glenville AR, et al. Distal Intestinal obstruction (DIOS) in patients with CF after lung transplantation. J Gastrointest Surg 2009; 13(8):1448-1453.
64. Koletzko S, Stringer DA, Cleghorn GJ, et al. Lavage treatment of DIOS in children with CF. Pediatrics 1989; 83:727-733.
65. Clifton IJ, Morton AM, Ambrose NS, et al. Treatment of resistant distal intestinal obstruction syndrome with a modified antegrade continence enema procedure. J Cyst Fibros 2004; 394:273-275.
66. Scott RB, O'Loughlin EV, Gall DG. Gastroesophageal reflux in patients with cystic fibrosis. J Pediatr 1985; 106(2):223-227.
67. Brodzicki J, Trawińska-Bartnicka M, Korzon M. Frequency, consequences and pharmacological treatment of gastroesophageal reflux in children with cystic fibrosis. Med Sci Monit 2002; 8(7):CR529-CR537.
68. Heine RG, Button BM, Olinsky A, et al. Gastro-oesophageal reflux in infants under 6 months with cystic fibrosis. Arch Dis Child 1998; 78(1):44-48.
69. Ledson MJ, Wilson GE, Tran J, et al. Tracheal microaspiration in adult cystic fibrosis. J R Soc Med 1998; 91(1):10-12.
70. Hallberg K, Fändriks L, Strandvik B. Duodenogastric bile reflux is common in cystic fibrosis. J Pediatr Gastroenterol Nutr 2004; 38(3):312-316.
71. Cucchiara S, Santamaria F, Andreotti MR, et al. Mechanisms of gastro-oesophageal reflux in cystic fibrosis. Arch Dis Child 1991; 66(5):617-622.
72. Orenstein SR, Khan S. Gastroesophageal reflux. In: Walker WA, Goulet O, Kleinman RE, et al., eds. Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, and Management. Hamilton Ontario (Canada): BC Decker, 2004:384-399.
73. Stringer DA, Sprigg A, Juodis E, et al. The association of cystic fibrosis, gastroesophageal reflux, and reduced pulmonary function. Can Assoc Radiol J 1988; 39(2):100-102.
74. Gustafsson PM, Fransson SG, Kjellman NI, et al. Gastro-oesophageal reflux and severity of pulmonary disease in cystic fibrosis. Scand J Gastroenterol 1991; 26(5):449-456.
75. Button BM, Heine RG, Catto-Smith AG, et al. Postural drainage in cystic fibrosis: is there a link with gastro-oesophageal reflux? J Paediatr Child Health 1998; 34(4):330-334.
76. Gregory PC. Gastrointestinal pH, motility/transit and permeability in cystic fibrosis. J Pediatr Gastroenterol Nutr 1996; 23(5):513-523.
77. Feigelson J, Girault F, Pecau Y. Gastro-oesophageal reflux and esophagitis in cystic fibrosis. Acta Paediatr Scand 1987; 76(6):989-990.
78. Hunt JF, Gaston B. Airway acidification and gastroesophageal reflux. Curr Allergy Asthma Rep 2008; 8(1):79-84.
79. Rosen R, Fritz J, Nurko A, et al. Lipid-laden macrophage index is not an indicator of gastroesophageal reflux-related respiratory disease in children. Pediatrics 2008; 121(4):e879-e884.
80. Starosta V, Kitz R, Hartl D, et al. Bronchoalveolar pepsin, bile acids, oxidation, and inflammation in children with gastroesophageal reflux disease. Chest 2007; 132(5):1557-1564.
81. Sweet MP, Patti MG, Hoopes C, et al. Gastro-oesophageal reflux and aspiration in patients with advanced lung disease. Thorax 2009; 64(2):167-173.
82. Farrell S, McMaster C, Gibson D, et al. Pepsin in bronchoalveolar lavage fluid: a specific and sensitive method of diagnosing gastro-oesophageal reflux-related pulmonary aspiration. J Pediatr Surg 2006; 41(2):289-293.
83. Button BM, Roberts S, Kotsimbos TC, et al. Gastroesophageal reflux (symptomatic and silent): a potentially significant problem in patients with cystic fibrosis before and after lung transplantation. J Heart Lung Transplant 2005; 24(10):1522-1529.
84. D'Ovidio F, Singer LG, Hadjiliadis D, et al. Prevalence of gastroesophageal reflux in endstage lung disease candidates for lung transplant. Ann Thorac Surg 2005; 80(4):1254-1260.
85. Benden C, Aurora P, Curry J, et al. High prevalence of gastroesophageal reflux in children after lung transplantation. Pediatr Pulmonol 2005; 40(1):68-71.
86. Young LR, Hadjiliadis D, Davis RD, et al. Lung transplantation exacerbates gastroesophageal reflux disease. Chest 2003; 124(5):1689-1693.
87. Hartwig MG, Appel JZ, Li B, et al. Chronic aspiration of gastric fluid accelerates pulmonary allograft dysfunction in a rat model of lung transplantation. J Thorac Cardiovasc Surg 2006; 131(1):209-217.
88. Ledson MJ, Tran J, Walshaw MJ. Prevalence and mechanisms of gastro-oesophageal reflux in adult cystic fibrosis patients. J R Soc Med 1998; 91(1):7-9.
89. James LP, Stowe CD, Farrar HC, et al. The pharmacokinetics of oral ranitidine in children and adolescents with cystic fibrosis. J Clin Pharmacol 1999; 39(12):1242-1247.
90. Tonelli AR, Drane WE, Collins DP, et al. Erythromycin improves gastric emptying halftime in adult cystic fibrosis patients with gastroparesis. J Cyst Fibros 2009; 8(3):193-197.
91. Boesch RP, Action JD. Outcomes of fundoplication in children with cystic fibrosis. J Pediatr Surg 2007; 42:1341-1344.
92. Palmer SM, Miralles AP, Howell DN, et al. Gastroesophageal reflux as a reversible cause of allograft dysfunction after lung transplantation. Chest 2000; 118(4):1214-1217.
93. Cantu E III, Appel JZ III, Hartwig MG, et al. Maxwell chamberlain memorial paper. Early fundoplication prevents chronic allograft dysfunction in patients with gastroesophageal reflux disease. Ann Thorac Surg 2004; 78(4):1142-1151.
94. Davis RD Jr., Lau CL, Eubanks S, et al. Improved lung allograft function after fundoplication in patients with gastroesophageal reflux disease undergoing lung transplantation. J Thorac Cardiovasc Surg 2003; 125(3):533-542.
95. Fridge JL, Conrad C, Gerson L, et al. Risk factors for small bowel bacterial overgrowth in cystic fibrosis. J Pediatr Gastroenterol Nutr 2007; 44(2):212-218.
96. Lewindon PJ, Robb TA, Moore DJ, et al. Bowel dysfunction in CF -Importance of breath testing. J Paediatr Child Health 1998; 34:79-82.
97. Schneider AR, Kluevber S, Posselt HG, et al. Application of the glucose hydrogen breath test for the detection of bacterial overgrowth in patients with cystic fibrosis-a reliable method? Dig Dis Sci 2008; [Epub ahead of print].
98. Wu TC, McCarthy VP, Gill VJ. Isolation rate and toxigenic potential of clostridium difficile isolates for patients with Cystic Fibrosis. J Infect Dis 1983; 148:176.
99. Theunissen C, Knoop C, NonhoffC, et al. Clostridium difficile colitis in cystic fibrosis patients with and without lung transplantation. Transpl Infect Dis 2008; 10(4):240-244.
100. Yates B, Murphy DM, Fisher AJ, et al. Pseudomembranous colitis in four patients with cystic fibrosis following lung transplantation. Thorax 2007; 62(6):554-556.
101. Yahav J, Samra Z, Blau H, et al. Helicobacter pylori and Clostridium difficile in cystic fibrosis patients. Dig Dis Sci 2006; 51(12):2274-2279.
102. Israel NR, Khanna B, Cutler A, et al. Seroprevalence of Helicobacter pylori infection in cystic fibrosis and its cross-reactivity with anti-pseudomonas antibodies. J Pediatr Gastroenterol Nutr 2000; 30(4):426-431.
103. Roberts DM, Craft JC, Mather FJ, et al. Prevalence of giardiasis in patients with cystic fibrosis. J Pediatr 1988; 112(4):555-559.
104. Rosenstein BJ, Perman JA, Kramer SS. Peptic ulcer disease in cystic fibrosis: an unusual occurrence in black adolescents. Am J Dis Child 1986; 140(10):966-969.
105. Kamath BM, Bhargave S, Markowitz JE, et al. Grand rounds: a girl with cystic fibrosis and failure to thrive. J Pediatr 2003; 143:115-19.
106. Fluge G, Olesen HV, Gilljam M, et al. Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients. J Cystic Fibros 2009; 8(3):198-202.
107. Lloyd-Still JD. Crohn's disease and cystic fibrosis. Dig Dis Sci 1994; 39(4)880-885.
108. Bresso F, Askling J, Astegiano M, et al. Potential role for the common cystic fibrosis Delta F508 mutation in Crohn's disease. Inflamm Bowel Dis 2006; 13(5):531-536.
109. Chaun H. Colonic disorders in adult cystic fibrosis. Can J Gastroenterol 2001; 15:586-590.
110. Condino AA, Hoffenberg EJ, Accurso F, et al. Frequency of ASCA seropositivity in children with cystic fibrosis. J Pediatr Gasteroenterol Nutr 2005; 41(1):23-26.
111. Wilchanski M, Kerem E, Adler S, et al. Capsule endoscopy in CF: the first look. Pediatr Pulmonol 2005; (suppl. 28):342.
112. Coughlin JP, Gauderer MW, Stern RC, et al. The spectrum of appendiceal disease in cystic fibrosis. J Pediatr Surg 1990; 25:835-839.
113. Sheilds MD, Levison H, Reisman JJ, et al. Appendicitis in cystic fibrosis. Arch Dis Child 1991; 66:307-310.
114. Lardenoye SW, Puylaert JB, Smit MJ, et al. Appendix in children with cystic fibrosis: US features. Radiology 2004; 232:187-189.
115. Friedlander JA, Mascarenhas MR, Garner LM, et al. Rectal Prolapse. Available at: http://emedicine.medscape.com/article/931455-overview.
116. Wilchanski M, Durie PR. Patterns of gastrointestinal disease in adulthood associated with mutations in CFTR gene. Gut 2007; 56:1153-1163.
117. Holsclaw DS, Rocmans C, Shwachman H. Intussuception in patients with cystic fibrosis. Pediatrics 1971; 48:51.
118. Pomerantz B, Anupindi S, Wales PW, et al. Radiographic reduction of intussuception in patients with cystic fibrosis. Pediatr Surg Int 2007; 23(8):763-765.
119. Collins CE, Francis JL, Thomas P, et al. Gastric emptying is faster in cystic fibrosis. J Pediatr Gastroenterol Nutr 1997; 25(5):492-498.
120. Cucchiara S, Raia V, Minella R. Ultrasound measurements of gastric emptying time in patients with cystic fibrosis and effect of ranitidine on delayed gastric emptying. J Pediatr 1996; 128(4):485-488.
121. Bodet-Milin C, Querellou S, Oudoux A, et al. Delayed gastric emptying scintigraphy in cystic fibrosis patients before and after lung transplantation. J Heart Lung Transplant 2006; 24(9):1077-1083.
122. Bentur L, Hino B, Shamir R, et al. Impaired gastric myoelectrical activity in patients with cystic fibrosis. J Cyst Fibros 2006; 5(3):187-191.
123. Rubinstein S, Moss R, Lewiston N. Constipation and meconium ileus equivalent in patients with CF. Pediatrics 1986; 78:473-479.
124. Delisle RC, Meldi L, Roach E, et al. Mast cells and gastrointestinal dysmotility in the cystic fibrosis mouse. PLos One 2009; 4(1):e4283; [doi:10.1371/journal.pone0004283].
125. Fitzsimmons SC, Burkhart GA, Borowitz D, et al. High dose pancreatic enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med 1997; 336:1283-1289.
126. Taylor CJ. Fibrosing colonopathy unrelated to pancreatic enzyme supplementation. J Pediatr Gastroenterol Nutr 2002; 35:3.
127. Lloyd-Still JD, Beno DW, Kimura RM. Cystic fibrosis colonopathy. Curr Gastrointest Rep 1999; 1:231-237.
128. Oermann CM, Al-Salmi Q, Seilheimer DK, et al. Mucinous cystadenocarcinoma of the pancreas in an adolescent with cystic fibrosis. Pediatr Dev Pathol 2005; 8(3):391-396.
129. McIntosh JC, Schoumacher RA, Tiller RE. Pancreatic adenocarcinoma in a patient with cystic fibrosis. Am J Med 1988; 85(4):592.
130. Tedesco FJ, Brown R, Schuman BM. Pancreatic carcinoma in a patient with cystic fibrosis. Gastrointest Endosc 1986; 32(1):25-26.
131. Neglia JP, FitzSimmons SC, Maisonneuve P, et al. The risk of cancer among patients with cystic fibrosis. Cystic Fibrosis and Cancer Study Group. N Engl J Med 1995; 332(8):494-499.
132. Sheldon CD, Hodson ME, Carpenter LM, et al. A cohort study of cystic fibrosis and malignancy. Br J Cancer 1993; 68(5):1025-1028.
133. Tsongalis GJ, Faber G, Dalldorf FG, et al. Association of pancreatic adenocarcinoma, mild lung disease, and delta F508 mutation in a cystic fibrosis patient. Clin Chem 1994; 40(10):1972-1974.
134. Petrowsky H, Schuster H, Irani S, et al Pancreatic cancer in cystic fibrosis after bilateral lung transplantation. Pancreas 2006; 33(4):430-432.
135. Maisonneuve P, Fitzsimmons SC, Neglia JP, et al. Cancer risk in non-transplanted and transplanted cystic fibrosis patients. J Natl Cancer Inst 2003; 95(5):381-387.
136. Davis TM, Sawicka EH. Adenocarcinoma in cystic fibrosis. Thorax 1985; 40(3):199-200.