The Journal of Pediatrics
Volume 151, Issue 3 , Pages 230-232 , September 2007

What is the Role of Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Primary Sclerosing Cholangitis?

  • Dennis D. Black

      Affiliations

    • Corresponding Author InformationReprint requests: Dennis D. Black, Department of Pediatrics, University of Tennessee Health Science Center, Children’s Foundation Research Center of Memphis, Le Bonheur Children’s Medical Center, Room 401, West Tower, 50 North Dunlap, Memphis, TN 38103.

References 

  1. LaRusso NF, Shneider BL, Black D, Gores GJ, James SP, Doo E, et al. Primary sclerosing cholangitis: summary of a workshop. Hepatology. 2006;44:746–764
  2. Feldstein AE, Perrault J, El-Youssif M, Lindor KD, Freese DK, Angulo P. Primary sclerosing cholangitis in children: a long-term follow-up study. Hepatology. 2003;38:210–217
  3. Olsson R, Boberg KM, de Muckadell OS, Lindgren S, Hultcrantz R, Folvik G, et al. High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study. Gastroenterology. 2005;129:1464–1472
  4. Gregorio GV, Portmann B, Karani J, Harrison P, Donaldson PT, Vergani D, et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology. 2001;33:544–553
  5. Lazaridis KN, Strazzabosco M, Larusso NF. The cholangiopathies: disorders of biliary epithelia. Gastroenterology. 2004;127:1565–1577
  6. Spirli C, Fabris L, Duner E, Fiorotto R, Ballardini G, Roskams T, et al. Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes. Gastroenterology. 2003;124:737–753
  7. Blanco PG, Zaman MM, Junaidi O, Sheth S, Yantiss RK, Nasser IA, et al. Induction of colitis in cftr−/− mice results in bile duct injury. Am J Physiol Gastrointest Liver Physiol. 2004;287:G491–G496
  8. Pall H, Zaman MM, Andersson C, Freedman SD. Decreased peroxisome proliferator activated receptor alpha is associated with bile duct injury in cystic fibrosis transmembrane conductance regulator−/− mice. J Pediatr Gastroenterol Nutr. 2006;42:275–281
  9. Beharry S, Ackerley C, Corey M, Kent G, Heng YM, Christensen H, et al. Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosis. Am J Physiol Gastrointest Liver Physiol. 2007;292:G839–G848
  10. McGill JM, Williams DM, Hunt CM. Survey of cystic fibrosis transmembrane conductance regulator genotypes in primary sclerosing cholangitis. Dig Dis Sci. 1996;41:540–542
  11. Girodon E, Sternberg D, Chazouilleres O, Cazeneuve C, Huot D, Calmus Y, et al. Cystic fibrosis transmembrane conductance regulator (CFTR) gene defects in patients with primary sclerosing cholangitis. J Hepatol. 2002;37:192–197
  12. Sheth S, Shea JC, Bishop MD, Chopra S, Regan MM, Malmberg E, et al. Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis. Hum Genet. 2003;113:286–292
  13. Gallegos-Orozco JF, C EY, Wang N, Rakela J, Charlton MR, Cutting GR, et al. Lack of association of common cystic fibrosis transmembrane conductance regulator gene mutations with primary sclerosing cholangitis. Am J Gastroenterol. 2005;100:874–878
  14. Pall H, Zielenski J, Jonas MM, Dasilva DA, Potvin KM, Yuan X-W, et al. Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function. J Pediatr. 2007;151:255–259
  15. Bresso F, Askling J, Astegiano M, Demarchi B, Sapone N, Rizzetto M, et al. Potential role for the common cystic fibrosis deltaF508 mutation in Crohn’s disease. Inflamm Bowel Dis. 2007;13:531–536

PII: S0022-3476(07)00486-6

doi: 10.1016/j.jpeds.2007.05.025

The Journal of Pediatrics
Volume 151, Issue 3 , Pages 230-232 , September 2007

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