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Published in: Digestive Diseases and Sciences 7/2015

01-07-2015 | Review

Gastrointestinal Manifestations of Cystic Fibrosis

Authors: Thomas Kelly, James Buxbaum

Published in: Digestive Diseases and Sciences | Issue 7/2015

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Abstract

Gastrointestinal symptoms of cystic fibrosis are the most important non-pulmonary manifestations of this genetic illness. Pancreatic manifestations include acute and chronic pancreatitis as well as pancreas insufficiency resulting in malnutrition. Complications in the gastrointestinal lumen are diverse and include distal intestinal obstruction syndrome (DIOS), meconium ileus, intussusception, and constipation; biliary tract complications include focal biliary cirrhosis and cholangiectasis. The common pathophysiology is the inspissation of secretions in the hollow structures of the gastrointestinal tract. Improved survival of CF patients mandates that the adult gastroenterologist be aware of the presentation and treatment of pancreatic, luminal, and hepatobiliary CF complications.
Literature
1.
go back to reference Gabriel SE, Brigman KN, Koller BH, et al. Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model. Science. 1994;266:107–109.PubMedCrossRef Gabriel SE, Brigman KN, Koller BH, et al. Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model. Science. 1994;266:107–109.PubMedCrossRef
5.
go back to reference Blackman SM, Deering-Brose R, McWilliams R, et al. Relative contribution of genetic and nongenetic modifiers to intestinal obstruction in cystic fibrosis. Gastroenterology. 2006;131:1030–1039.PubMedCentralPubMedCrossRef Blackman SM, Deering-Brose R, McWilliams R, et al. Relative contribution of genetic and nongenetic modifiers to intestinal obstruction in cystic fibrosis. Gastroenterology. 2006;131:1030–1039.PubMedCentralPubMedCrossRef
6.
go back to reference Kopelman H, Durie P, Gaskin K, et al. Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. N Engl J Med. 1985;312:329–334.PubMedCrossRef Kopelman H, Durie P, Gaskin K, et al. Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. N Engl J Med. 1985;312:329–334.PubMedCrossRef
7.
go back to reference Shwachman H, Lebenthal E, Khaw KT. Recurrent acute pancreatitis in patients with cystic fibrosis with normal pancreatic enzymes. Pediatrics. 1975;55:86–95.PubMed Shwachman H, Lebenthal E, Khaw KT. Recurrent acute pancreatitis in patients with cystic fibrosis with normal pancreatic enzymes. Pediatrics. 1975;55:86–95.PubMed
8.
go back to reference De Boeck K, Weren M, Proesmans M, et al. Pancreatitis among patients with cystic fibrosis: correlation with pancreatic status and genotype. Pediatrics. 2005;115:e463–e469.PubMedCrossRef De Boeck K, Weren M, Proesmans M, et al. Pancreatitis among patients with cystic fibrosis: correlation with pancreatic status and genotype. Pediatrics. 2005;115:e463–e469.PubMedCrossRef
9.
go back to reference King LJ, Scurr ED, Murugan N, et al. Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances. Radiographics. 2000;20:767–777.PubMedCrossRef King LJ, Scurr ED, Murugan N, et al. Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances. Radiographics. 2000;20:767–777.PubMedCrossRef
10.
go back to reference Sharer N, Schwarz M, Malone G, et al. Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N Engl J Med. 1998;339:645–652.PubMedCrossRef Sharer N, Schwarz M, Malone G, et al. Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N Engl J Med. 1998;339:645–652.PubMedCrossRef
11.
go back to reference 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;322:303–308.PubMedCrossRef 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;322:303–308.PubMedCrossRef
12.
go back to reference 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;162:760–763.PubMedCrossRef 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;162:760–763.PubMedCrossRef
13.
go back to reference Dodge JA, Turck D. Cystic fibrosis: nutritional consequences and management. Best Pract Res Clin Gastroenterol. 2006;20:531–546.PubMedCrossRef Dodge JA, Turck D. Cystic fibrosis: nutritional consequences and management. Best Pract Res Clin Gastroenterol. 2006;20:531–546.PubMedCrossRef
14.
go back to reference Agrons GA, Corse WR, Markowitz RI, et al. Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. Radiographics. 1996;16:871–893.PubMedCrossRef Agrons GA, Corse WR, Markowitz RI, et al. Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. Radiographics. 1996;16:871–893.PubMedCrossRef
15.
go back to reference Liong SY, Awad D, Jones AM, et al. The adult cystic fibrosis patient with abdominal pain: what the radiologist needs to know. Clin Radiol. 2011;66:132–139.PubMedCrossRef Liong SY, Awad D, Jones AM, et al. The adult cystic fibrosis patient with abdominal pain: what the radiologist needs to know. Clin Radiol. 2011;66:132–139.PubMedCrossRef
16.
go back to reference Chaudry G, Navarro OM, Levine DS, et al. Abdominal manifestations of cystic fibrosis in children. Pediatr Radiol. 2006;36:233–240.PubMedCrossRef Chaudry G, Navarro OM, Levine DS, et al. Abdominal manifestations of cystic fibrosis in children. Pediatr Radiol. 2006;36:233–240.PubMedCrossRef
17.
go back to reference Andersen D. Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathologic study. Am J Dis Chil. 1938;56:344–399.CrossRef Andersen D. Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathologic study. Am J Dis Chil. 1938;56:344–399.CrossRef
18.
19.
go back to reference Mousa HM, Woodley FW. Gastroesophageal reflux in cystic fibrosis: current understandings of mechanisms and management. Curr Gastroenterol Rep. 2012;14:226–235.PubMedCrossRef Mousa HM, Woodley FW. Gastroesophageal reflux in cystic fibrosis: current understandings of mechanisms and management. Curr Gastroenterol Rep. 2012;14:226–235.PubMedCrossRef
20.
21.
go back to reference Orenstein SR, Orenstein DM. Gastroesophageal reflux and respiratory disease in children. J Pediatr. 1988;112:847–858.PubMedCrossRef Orenstein SR, Orenstein DM. Gastroesophageal reflux and respiratory disease in children. J Pediatr. 1988;112:847–858.PubMedCrossRef
22.
go back to reference Eggermont E, Deboeck K. Small-intestinal abnormalities in cystic fibrosis patients. Eur J Pediatr. 1991;150:824–828.PubMedCrossRef Eggermont E, Deboeck K. Small-intestinal abnormalities in cystic fibrosis patients. Eur J Pediatr. 1991;150:824–828.PubMedCrossRef
23.
go back to reference Constantine S, Au VW, Slavotinek JP. Abdominal manifestations of cystic fibrosis in adults: a review. Australas Radiol. 2004;48:450–458.PubMedCrossRef Constantine S, Au VW, Slavotinek JP. Abdominal manifestations of cystic fibrosis in adults: a review. Australas Radiol. 2004;48:450–458.PubMedCrossRef
24.
go back to reference Grossman H, Berdon WE, Baker DH. Gastrointestinal findings in cystic fibrosis. Am J Roentgenol Radium Ther Nucl Med. 1966;97:227–238.PubMedCrossRef Grossman H, Berdon WE, Baker DH. Gastrointestinal findings in cystic fibrosis. Am J Roentgenol Radium Ther Nucl Med. 1966;97:227–238.PubMedCrossRef
25.
go back to reference Abramson SJ, Baker DH, Amodio JB, et al. Gastrointestinal manifestations of cystic fibrosis. Semin Roentgenol. 1987;22:97–113.PubMedCrossRef Abramson SJ, Baker DH, Amodio JB, et al. Gastrointestinal manifestations of cystic fibrosis. Semin Roentgenol. 1987;22:97–113.PubMedCrossRef
26.
go back to reference Robertson MB, Choe KA, Joseph PM. Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 2006;26:679–690.PubMedCrossRef Robertson MB, Choe KA, Joseph PM. Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 2006;26:679–690.PubMedCrossRef
27.
go back to reference Phelan MS, Fine DR, Zentlermunro PL, et al. Radiographic abnormalities of the duodenum in cystic fibrosis. Clin Radiol. 1983;34:573–577.PubMedCrossRef Phelan MS, Fine DR, Zentlermunro PL, et al. Radiographic abnormalities of the duodenum in cystic fibrosis. Clin Radiol. 1983;34:573–577.PubMedCrossRef
28.
29.
30.
go back to reference Gorter RR, Karimi A, Sleeboom C, et al. Clinical and genetic characteristics of meconium ileus in newborns with and without cystic fibrosis. J Pediatr Gastroenterol Nutr. 2010;50:569–572.PubMed Gorter RR, Karimi A, Sleeboom C, et al. Clinical and genetic characteristics of meconium ileus in newborns with and without cystic fibrosis. J Pediatr Gastroenterol Nutr. 2010;50:569–572.PubMed
32.
go back to reference Wyllie R. Gastrointestinal manifestations of cystic fibrosis. Clin Pediatr. 1999;38:735–738.CrossRef Wyllie R. Gastrointestinal manifestations of cystic fibrosis. Clin Pediatr. 1999;38:735–738.CrossRef
33.
go back to reference Casaccia G, Trucchi A, Nahom A, et al. The impact of cystic fibrosis on neonatal intestinal obstruction: the need for prenatal/neonatal screening. Pediatr Surg Int. 2003;19:75–78.PubMed Casaccia G, Trucchi A, Nahom A, et al. The impact of cystic fibrosis on neonatal intestinal obstruction: the need for prenatal/neonatal screening. Pediatr Surg Int. 2003;19:75–78.PubMed
34.
go back to reference Lang I, Daneman A, Cutz E, et al. Abdominal calcification in cystic fibrosis with meconium ileus: radiologic-pathologic correlation. Pediatr Radiol. 1997;27:523–527.PubMedCrossRef Lang I, Daneman A, Cutz E, et al. Abdominal calcification in cystic fibrosis with meconium ileus: radiologic-pathologic correlation. Pediatr Radiol. 1997;27:523–527.PubMedCrossRef
35.
go back to reference Delpin CA, Czyrko C, Ziegler MM, et al. Management and survival of meconium ileus—A 30-year review. Ann Surg. 1992;215:179–185.CrossRef Delpin CA, Czyrko C, Ziegler MM, et al. Management and survival of meconium ileus—A 30-year review. Ann Surg. 1992;215:179–185.CrossRef
36.
go back to reference Colombo C, Ellemunter H, Houwen R, et al. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. J Cyst Fibros. 2011;10:S24–S28.PubMedCrossRef Colombo C, Ellemunter H, Houwen R, et al. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. J Cyst Fibros. 2011;10:S24–S28.PubMedCrossRef
37.
go back to reference Dray X, Bienvenu T, Desmazes-Dufeu N, et al. Distal intestinal obstruction syndrome in adults with cystic fibrosis. Clin Gastroenterol Hepatol. 2004;2:498–503.PubMedCrossRef Dray X, Bienvenu T, Desmazes-Dufeu N, et al. Distal intestinal obstruction syndrome in adults with cystic fibrosis. Clin Gastroenterol Hepatol. 2004;2:498–503.PubMedCrossRef
38.
go back to reference Houwen RH, van der Doef HP, Sermet I, et al. Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS. J Pediatr Gastroenterol Nutr. 2010;50:38–42.PubMedCrossRef Houwen RH, van der Doef HP, Sermet I, et al. Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS. J Pediatr Gastroenterol Nutr. 2010;50:38–42.PubMedCrossRef
39.
40.
go back to reference van der Doef HP, Kokke FT, van der Ent CK, et al. Intestinal obstruction syndromes in cystic fibrosis: meconium ileus, distal. Curr Gastroenterol Rep. 2011;13:265–270.PubMedCentralPubMedCrossRef van der Doef HP, Kokke FT, van der Ent CK, et al. Intestinal obstruction syndromes in cystic fibrosis: meconium ileus, distal. Curr Gastroenterol Rep. 2011;13:265–270.PubMedCentralPubMedCrossRef
41.
go back to reference Stern RC, Izant RJ, Boat TF, et al. Treatment and prognosis of rectal prolapse in cystic-fibrosis. Gastroenterology. 1982;82:707–710.PubMed Stern RC, Izant RJ, Boat TF, et al. Treatment and prognosis of rectal prolapse in cystic-fibrosis. Gastroenterology. 1982;82:707–710.PubMed
42.
go back to reference Siafakas C, Vottler TP, Andersen JM. Rectal prolapse in pediatrics. Clin Pediatr. 1999;38:63–72.CrossRef Siafakas C, Vottler TP, Andersen JM. Rectal prolapse in pediatrics. Clin Pediatr. 1999;38:63–72.CrossRef
43.
go back to reference Nash EF, Stephenson A, Helm EJ, et al. Intussusception in adults with cystic fibrosis: a case series with review of the literature. Dig Dis Sci. 2011;56:3695–3700.PubMedCrossRef Nash EF, Stephenson A, Helm EJ, et al. Intussusception in adults with cystic fibrosis: a case series with review of the literature. Dig Dis Sci. 2011;56:3695–3700.PubMedCrossRef
44.
go back to reference Munck A, Belarbi N, de Lagausie P, et al. Ultrasonography detects appendicular mucocele in cystic fibrosis patients suffering recurrent abdominal pain. Pediatrics. 2000;105:921.PubMedCrossRef Munck A, Belarbi N, de Lagausie P, et al. Ultrasonography detects appendicular mucocele in cystic fibrosis patients suffering recurrent abdominal pain. Pediatrics. 2000;105:921.PubMedCrossRef
45.
go back to reference Coughlin JP, Gauderer MWL, Stern RC, et al. The spectrum of appendiceal disease in cystic fibrosis. J Pediatr Surg. 1990;25:835–839.PubMedCrossRef Coughlin JP, Gauderer MWL, Stern RC, et al. The spectrum of appendiceal disease in cystic fibrosis. J Pediatr Surg. 1990;25:835–839.PubMedCrossRef
46.
go back to reference Lardenoye SW, Puylaert JB, Smit MJ, et al. Appendix in children with cystic fibrosis: US features. Radiology. 2004;232:187–189.PubMedCrossRef Lardenoye SW, Puylaert JB, Smit MJ, et al. Appendix in children with cystic fibrosis: US features. Radiology. 2004;232:187–189.PubMedCrossRef
47.
go back to reference Chaun H. Colonic disorders in adult cystic fibrosis. Can J Gastroenterol. 2001;15:586–590.PubMed Chaun H. Colonic disorders in adult cystic fibrosis. Can J Gastroenterol. 2001;15:586–590.PubMed
48.
go back to reference Smyth RL, Vanvelzen D, Smyth AR, et al. Strictures of ascending colon in cystic fibrosis and high-strength pancreatic-enzymes. Lancet. 1994;343:85–86.PubMedCrossRef Smyth RL, Vanvelzen D, Smyth AR, et al. Strictures of ascending colon in cystic fibrosis and high-strength pancreatic-enzymes. Lancet. 1994;343:85–86.PubMedCrossRef
49.
go back to reference Smyth RL, Ashby D, Ohea U, et al. Fibrosing colonopathy in cystic fibrosis—results of a case-control study. Lancet. 1995;346:1247–1251.PubMedCrossRef Smyth RL, Ashby D, Ohea U, et al. Fibrosing colonopathy in cystic fibrosis—results of a case-control study. Lancet. 1995;346:1247–1251.PubMedCrossRef
50.
go back to reference Moss RL, Musemeche CA, Feddersen RM. Progressive pan-colonic fibrosis secondary to oral administration of pancreatic enzymes. Pediatr Surg Int. 1998;13:168–170.PubMedCrossRef Moss RL, Musemeche CA, Feddersen RM. Progressive pan-colonic fibrosis secondary to oral administration of pancreatic enzymes. Pediatr Surg Int. 1998;13:168–170.PubMedCrossRef
51.
go back to reference Schneider ARJ, Klueber 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. 2009;54:1730–1735.PubMedCrossRef Schneider ARJ, Klueber 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. 2009;54:1730–1735.PubMedCrossRef
52.
go back to reference 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;332:494–499.PubMedCrossRef 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;332:494–499.PubMedCrossRef
53.
go back to reference Meyer KC, Francois ML, Thomas HK, et al. Colon cancer in lung transplant recipients with CF: increased risk and results of screening. J Cyst Fibros. 2011;10:366–369.PubMedCrossRef Meyer KC, Francois ML, Thomas HK, et al. Colon cancer in lung transplant recipients with CF: increased risk and results of screening. J Cyst Fibros. 2011;10:366–369.PubMedCrossRef
54.
go back to reference Feranchak AP. Hepatobiliary complications of cystic fibrosis. Curr Gastroenterol Rep. 2004;6:231–239.PubMedCrossRef Feranchak AP. Hepatobiliary complications of cystic fibrosis. Curr Gastroenterol Rep. 2004;6:231–239.PubMedCrossRef
55.
go back to reference Sokol RJ, Durie PR. Cystic Fibrosis Fdn Hepatobiliary Dis C. Recommendations for management of liver and biliary tract disease in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1999;28:S1–S13.PubMedCrossRef Sokol RJ, Durie PR. Cystic Fibrosis Fdn Hepatobiliary Dis C. Recommendations for management of liver and biliary tract disease in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1999;28:S1–S13.PubMedCrossRef
57.
go back to reference Cohn JA, Strong TV, Picciotto MR, et al. Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells. Gastroenterology. 1993;105:1857–1864.PubMed Cohn JA, Strong TV, Picciotto MR, et al. Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells. Gastroenterology. 1993;105:1857–1864.PubMed
58.
go back to reference Kochar R, Fallon MB. Pulmonary diseases and the liver. Clin Liver Dis. 2011;15:21. Kochar R, Fallon MB. Pulmonary diseases and the liver. Clin Liver Dis. 2011;15:21.
59.
go back to reference Herrmann U, Dockter G, Lammert F. Cystic fibrosis-associated liver disease. Best Pract Res Clin Gastroenterol. 2010;24:585–592.PubMedCrossRef Herrmann U, Dockter G, Lammert F. Cystic fibrosis-associated liver disease. Best Pract Res Clin Gastroenterol. 2010;24:585–592.PubMedCrossRef
60.
go back to reference Lewindon PJ, Pereira TN, Hoskins AC, et al. The role of hepatic stellate cells and transforming growth factor-beta(1) in cystic fibrosis liver disease. Am J Pathol. 2002;160:1705–1715.PubMedCentralPubMedCrossRef Lewindon PJ, Pereira TN, Hoskins AC, et al. The role of hepatic stellate cells and transforming growth factor-beta(1) in cystic fibrosis liver disease. Am J Pathol. 2002;160:1705–1715.PubMedCentralPubMedCrossRef
61.
go back to reference Lindblad A, Glaumann H, Strandvik B. Natural history of liver disease in cystic fibrosis. Hepatology. 1999;30:1151–1158.PubMedCrossRef Lindblad A, Glaumann H, Strandvik B. Natural history of liver disease in cystic fibrosis. Hepatology. 1999;30:1151–1158.PubMedCrossRef
62.
go back to reference Colombo C, Battezzati PM, Crosignani A, et al. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors, and outcome. Hepatology. 2002;36:1374–1382.PubMedCrossRef Colombo C, Battezzati PM, Crosignani A, et al. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors, and outcome. Hepatology. 2002;36:1374–1382.PubMedCrossRef
63.
go back to reference Bartlett JR, Friedman KJ, Ling SC, et al. Genetic modifiers of liver disease in cystic fibrosis. J Am Med Assoc. 2009;302:1076–1083.CrossRef Bartlett JR, Friedman KJ, Ling SC, et al. Genetic modifiers of liver disease in cystic fibrosis. J Am Med Assoc. 2009;302:1076–1083.CrossRef
64.
go back to reference Lindblad A, Glaumann H, Strandvik B. A two-year prospective study of the effect of ursodeoxycholic acid on urinary bile acid excretion and liver morphology in cystic fibrosis-associated liver disease. Hepatology. 1998;27:166–174.PubMedCrossRef Lindblad A, Glaumann H, Strandvik B. A two-year prospective study of the effect of ursodeoxycholic acid on urinary bile acid excretion and liver morphology in cystic fibrosis-associated liver disease. Hepatology. 1998;27:166–174.PubMedCrossRef
65.
go back to reference Colombo C, Crosignani A, Assaisso M, et al. Ursodeoxycholic acid therapy in cystic fibrosis-associated liver disease: a dose-response study. Hepatology. 1992;16:924–930.PubMedCrossRef Colombo C, Crosignani A, Assaisso M, et al. Ursodeoxycholic acid therapy in cystic fibrosis-associated liver disease: a dose-response study. Hepatology. 1992;16:924–930.PubMedCrossRef
66.
go back to reference Colombo C, Battezzati PM, Podda M, et al. Ursodeoxycholic acid for liver disease associated with cystic fibrosis: a double-mind multicenter trial. Hepatology. 1996;23:1484–1490.PubMedCrossRef Colombo C, Battezzati PM, Podda M, et al. Ursodeoxycholic acid for liver disease associated with cystic fibrosis: a double-mind multicenter trial. Hepatology. 1996;23:1484–1490.PubMedCrossRef
67.
go back to reference Cheng K, Ashby D, Smyth RL. Ursodeoxycholic acid for cystic fibrosis-related liver disease. Cochrane Database Syst Rev. 2012;10:CD000222. Cheng K, Ashby D, Smyth RL. Ursodeoxycholic acid for cystic fibrosis-related liver disease. Cochrane Database Syst Rev. 2012;10:CD000222.
68.
go back to reference Imam MH, Sinakos E, Gossard AA, et al. High-dose ursodeoxycholic acid increases risk of adverse outcomes in patients with early stage primary sclerosing cholangitis. Aliment Pharmacol Ther. 2011;34:1185–1192.PubMedCentralPubMedCrossRef Imam MH, Sinakos E, Gossard AA, et al. High-dose ursodeoxycholic acid increases risk of adverse outcomes in patients with early stage primary sclerosing cholangitis. Aliment Pharmacol Ther. 2011;34:1185–1192.PubMedCentralPubMedCrossRef
69.
go back to reference Lindor KD, Kowdley KV, Luketic VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology. 2009;50:808–814.PubMedCentralPubMedCrossRef Lindor KD, Kowdley KV, Luketic VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology. 2009;50:808–814.PubMedCentralPubMedCrossRef
70.
go back to reference Lu BR, Esquivel CO. A review of abdominal organ transplantation in cystic fibrosis. Pediatr Transpl. 2010;14:954–960.CrossRef Lu BR, Esquivel CO. A review of abdominal organ transplantation in cystic fibrosis. Pediatr Transpl. 2010;14:954–960.CrossRef
71.
go back to reference Molmenti EP, Squires RH, Nagata D, et al. Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population. Pediatr Transpl. 2003;7:93–97.CrossRef Molmenti EP, Squires RH, Nagata D, et al. Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population. Pediatr Transpl. 2003;7:93–97.CrossRef
72.
go back to reference Enriquez G, Lucaya J, Allende E, et al. Intrahepatic biliary stone in children. Pediatr Radiol. 1992;22:283–286.PubMedCrossRef Enriquez G, Lucaya J, Allende E, et al. Intrahepatic biliary stone in children. Pediatr Radiol. 1992;22:283–286.PubMedCrossRef
73.
go back to reference Gaskin KJ, Waters DLM, Howmangiles R, et al. Liver disease and common-bile-duct stenosis in cystic fibrosis. N Engl J Med. 1988;318:340–346.PubMedCrossRef Gaskin KJ, Waters DLM, Howmangiles R, et al. Liver disease and common-bile-duct stenosis in cystic fibrosis. N Engl J Med. 1988;318:340–346.PubMedCrossRef
74.
go back to reference Perdue DG, Cass OW, Milla C, et al. Hepatolithiasis and Cholangiocarcinoma in cystic fibrosis: a case series and review of the literature. Dig Dis Sci. 2007;52:2638–2642.PubMedCrossRef Perdue DG, Cass OW, Milla C, et al. Hepatolithiasis and Cholangiocarcinoma in cystic fibrosis: a case series and review of the literature. Dig Dis Sci. 2007;52:2638–2642.PubMedCrossRef
75.
go back to reference Bass S, Connon JJ, Ho CS. Biliary tree in cystic fibrosis: biliary tract abnormalities in cystic fibrosis demonstrated by endoscopic retrograde cholangiography. Gastroenterology. 1983;84:1592–1596.PubMed Bass S, Connon JJ, Ho CS. Biliary tree in cystic fibrosis: biliary tract abnormalities in cystic fibrosis demonstrated by endoscopic retrograde cholangiography. Gastroenterology. 1983;84:1592–1596.PubMed
76.
go back to reference Baldwin TL, Schutz SM, Owens G, et al. Successful endoscopic therapy of cholangitis associated with intrahepatic cholangiectasis in adult cystic fibrosis. Gastrointest Endosc. 1999;49:249–251.PubMedCrossRef Baldwin TL, Schutz SM, Owens G, et al. Successful endoscopic therapy of cholangitis associated with intrahepatic cholangiectasis in adult cystic fibrosis. Gastrointest Endosc. 1999;49:249–251.PubMedCrossRef
77.
go back to reference Lo CM, Fan ST, Wong J. The changing epidemiology of recurrent pyogenic cholangitis. Hong Kong Med J. 1997;3:302–304.PubMed Lo CM, Fan ST, Wong J. The changing epidemiology of recurrent pyogenic cholangitis. Hong Kong Med J. 1997;3:302–304.PubMed
79.
go back to reference Wasmuth HE, Keppeler H, Herrmann U, et al. Coinheritance of Gilbert syndrome-associated UGT1A1 mutation increases gallstone risk in cystic fibrosis. Hepatology. 2006;43:738–741.PubMedCrossRef Wasmuth HE, Keppeler H, Herrmann U, et al. Coinheritance of Gilbert syndrome-associated UGT1A1 mutation increases gallstone risk in cystic fibrosis. Hepatology. 2006;43:738–741.PubMedCrossRef
80.
go back to reference Karlas T, Neuschulz M, Oltmanns A, et al. Non-invasive evaluation of cystic fibrosis related liver disease in adults with ARFI, transient elastography and different fibrosis scores. Plos One. 2012;7:8.CrossRef Karlas T, Neuschulz M, Oltmanns A, et al. Non-invasive evaluation of cystic fibrosis related liver disease in adults with ARFI, transient elastography and different fibrosis scores. Plos One. 2012;7:8.CrossRef
81.
go back to reference Treem WR, Stanley CA. Massive hepatomegaly, steatosis, and secondary plasma carnitine deficiency in an infant with cystic fibrosis. Pediatrics. 1989;83:993–997.PubMed Treem WR, Stanley CA. Massive hepatomegaly, steatosis, and secondary plasma carnitine deficiency in an infant with cystic fibrosis. Pediatrics. 1989;83:993–997.PubMed
82.
go back to reference Aldamiz-Echevarria L, Prieto J, Andrade F, et al. Persistence of essential fatty acid deficiency in cystic fibrosis despite nutritional therapy. Pediatr Res. 2009;66:585–589.PubMedCrossRef Aldamiz-Echevarria L, Prieto J, Andrade F, et al. Persistence of essential fatty acid deficiency in cystic fibrosis despite nutritional therapy. Pediatr Res. 2009;66:585–589.PubMedCrossRef
83.
go back to reference Freedman SD, Shea JC, Blanco PG, et al. Fatty acids in cystic fibrosis. Curr Opin Pulm Med. 2000;6:530–532.PubMedCrossRef Freedman SD, Shea JC, Blanco PG, et al. Fatty acids in cystic fibrosis. Curr Opin Pulm Med. 2000;6:530–532.PubMedCrossRef
84.
go back to reference Colombo C, Bertolini E, Assaisso ML, et al. Failure of ursodeoxycholic acid to dissolve radiolucent gallstones in patients with cystic fibrosis. Acta Paediatr. 1993;82:562–565.PubMedCrossRef Colombo C, Bertolini E, Assaisso ML, et al. Failure of ursodeoxycholic acid to dissolve radiolucent gallstones in patients with cystic fibrosis. Acta Paediatr. 1993;82:562–565.PubMedCrossRef
85.
go back to reference Angelico M, Gandin C, Canuzzi P, et al. Gallstones in cystic fibrosis: a critical reappraisal. Hepatology. 1991;14:768–775.PubMedCrossRef Angelico M, Gandin C, Canuzzi P, et al. Gallstones in cystic fibrosis: a critical reappraisal. Hepatology. 1991;14:768–775.PubMedCrossRef
86.
go back to reference Roy CC, Weber AM, Morin CL, et al. Abnormal biliary lipid composition in cystic fibrosis: effect of pancreatic enzymes. N Engl J Med. 1977;297:1301–1305.PubMedCrossRef Roy CC, Weber AM, Morin CL, et al. Abnormal biliary lipid composition in cystic fibrosis: effect of pancreatic enzymes. N Engl J Med. 1977;297:1301–1305.PubMedCrossRef
Metadata
Title
Gastrointestinal Manifestations of Cystic Fibrosis
Authors
Thomas Kelly
James Buxbaum
Publication date
01-07-2015
Publisher
Springer US
Published in
Digestive Diseases and Sciences / Issue 7/2015
Print ISSN: 0163-2116
Electronic ISSN: 1573-2568
DOI
https://doi.org/10.1007/s10620-015-3546-7

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