Current Gastroenterology Reports

, Volume 14, Issue 3, pp 226–235

Gastroesophageal Reflux in Cystic Fibrosis: Current Understandings of Mechanisms and Management

Pediatric Gastroenterology (SR Orenstein, Section Editor)


Cystic fibrosis (CF) is an inherited disease that affects both the lungs and the digestive system in children and adults. Thick mucus fills the gut and blocks lumens of the pancreas and hepatobiliary systems, creating insufficient pancreas function and liver disease. Chronic gastrointestinal (GI) complications, including intestinal obstruction, occur in neonates, and poor digestion and gastroesophageal reflux disease (GERD) in children. Although GI symptoms tend to improve with age, CF and associated GERD eventually create respiratory insufficiency; the only available treatment option at this stage is a bilateral lung transplant, which carries considerable morbidity and mortality. While GERD may reoccur as a complication of lung transplantation, GERD symptoms are often reduced following a fundoplication.


Children Chronic cough Cystic fibrosis Gastric hyperacidity Fundoplication GER (gastroesophageal reflux) Impedance (LES) lower esophageal sphincter pH monitoring 


Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. 1.
    • Kaitlin JP, Schultz K. Cystic fibrosis: overview of gastrointestinal disease. UpToDate. May 2011, version 19.2. This paper from Kaitlin and Schultz provides an in depth overview of gastrointestinal complications as they relate to increasing morbidity in patients with cystic fibrosis. Topic review includes intestinal abnormalities (such as gastroesophageal reflux disease, meconium ileus, distal intestinal obstruction syndrome, intussusception, small intestine bacterial overgrowth, constipation, and rectal prolapse), pancreatic insufficiency, and hepatobiliary disease. Google Scholar
  2. 2.
    Brodzicki J, Trawinska M, Korzon M. Frequency, consequences and pharmacological treatment of gastroesophageal reflux in children with cystic fibrosis. Med Sci Monit. 2002;8:529–37.Google Scholar
  3. 3.
    Milla PJ. Cystic fibrosis: present and future. Digestion. Eur J Gastroenterol Hepatol. 1996;59:579–88.Google Scholar
  4. 4.
    Rozmanic V, Tjesic-Drinkovic D, Banac S, et al. Gastroesophageal reflux and gastric hyperacidity in cystic fibrosis patients. Pedijatrija danas. 2010;6:45–52.Google Scholar
  5. 5.
    Gregory PC. Gastrointestinal pH, motility/transit and permeability in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1996;23:513–23.PubMedCrossRefGoogle Scholar
  6. 6.
    Malfroot A, Dab I. New insights on gastro-oesophageal reflux in cystic fibrosis by longitudinal follow up. Arch Dis Child. 1991;66:1339–45.PubMedCrossRefGoogle Scholar
  7. 7.
    Gustafsson PM, Fransson SG, Kuellman M, et al. Gastro-oesophageal reflux and severity of pulmonary disease in cystic fibrosis. Scan J Gastroenterol. 1991;26:449–56.CrossRefGoogle Scholar
  8. 8.
    • Blondeau K, Pauwels A, Dupont LJ, et al. Characteristics of gastroesophageal reflux and potential risk of gastric content aspiration in children with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2010;50(2):161–6. Blondeau et al. use combined esophageal pH monitoring and multichannel intraluminal impedance to assess the characteristics of GER (acidic, weakly acidic, and weakly alkaline) in children with cystic fibrosis. In this study, Blondeau and colleagues find that GER is a primary phenomenon in cystic fibrosis patients and not secondary to cough. PubMedCrossRefGoogle Scholar
  9. 9.
    Cucchiara S, Raia V, Minella R, et al. Ultrasound measurement of gastric emptying time in patients with cystic fibrosis and effect of ranitidine on delayed gastric emptying. J Pediatr. 1996;128:485–8.PubMedCrossRefGoogle Scholar
  10. 10.
    Button BM, Heine RG, Catto-Smith AG, et al. Postural drainage and gastro-oesophageal reflux in infants with cystic fibrosis. Arch Dis Child. 1997;76:148–50.PubMedCrossRefGoogle Scholar
  11. 11.
    Dab I, Malfroot A. Gastroesophageal reflux: a primary defect in cystic fibrosis? Scan J Gastroenterol. 1988;143:125–31.CrossRefGoogle Scholar
  12. 12.
    Fathi H, Moon I, Donaldson J. Cough in adult cystic fibrosis: diagnosis and response to fundoplication. Cough. 2009;5:1–6.PubMedCrossRefGoogle Scholar
  13. 13.
    Ledson MJ, Tran J, Walshaw MJ. Prevalence and mechanisms of gastro-oesophageal reflux in adult cystic fibrosis patients. J R Soc Med. 1996;91:7–9.Google Scholar
  14. 14.
    Cucchiara S, Santamaria F, Andreotti MR. Mechanisms of gastro-oesophageal reflux in cystic fibrosis. Arch Dis Child. 1991;66:617–22.PubMedCrossRefGoogle Scholar
  15. 15.
    • 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 Trans. 2005;24:1522–9. Using esophageal pH monitoring with a dual pH probe, Button et al. found that GER (both the symptomatic and the silent) is significant in CF patients and contributes to declining lung function through microaspiration and reflux bronchospasm. The authors also show that pre-existing GER is exacerbated following lung replacement. CrossRefGoogle Scholar
  16. 16.
    Bontempo I, Piretta L, Corazziari E, et al. Effects of intraluminal acidification on oesophageal motor activity. Gut. 1994;35:884–90.PubMedCrossRefGoogle Scholar
  17. 17.
    Hodson ME, Geddes DM, editors. Cystic fibrosis. London: Chapman & Hall; 1995.Google Scholar
  18. 18.
    Ledson MJ, Wilson GE, Tran J, et al. Tracheal microaspiration in adult cystic fibrosis. J R Soc Med. 1998;91:10–2.PubMedGoogle Scholar
  19. 19.
    Gilljam J, Chaparro C, Tullise E, et al. GI complications after lung transplantation in patients with cystic fibrosis. Chest. 2003;1:37–41.CrossRefGoogle Scholar
  20. 20.
    Young LR, Hadjiliadis D, Davis RD, et al. Lung transplantation exacerbates gastroesophageal reflux disease. Chest. 2003;5:1689–93.CrossRefGoogle Scholar
  21. 21.
    Eggermont E. Gastrointestinal manifestations in cystic fibrosis. Eur J Gastroenterol Hepatol. 1996;8:731–8.PubMedGoogle Scholar
  22. 22.
    • Blondeau K, Mertens V, Vanaudenaerde BA, et al. Gastro-oesophageal reflux and gastric aspiration in lung transplant patients with or without chronic rejection. Eur Respir J. 2008;31:707–13. Blondeau and colleagues are the first to report the use of combined esophageal pH monitoring and multichannel intraluminal impedance to characterize the frequency and type (acid and non-acid) of GER in patients with cystic fibrosis. Also, in this study, Blondeau and colleagues found that treatment with PPI did not reduce the levels of pepsin and bile in bronchoalveolar lavage fluid. PubMedCrossRefGoogle Scholar
  23. 23.
    Palmer SM, Miralles AP, Howell DN, et al. Gastroesophageal reflux as a reversible cause of allograft dysfunction after lung trasnplantation. Chest. 2000;118:1214–7.PubMedCrossRefGoogle Scholar
  24. 24.
    Verleden GM, Dupont LJ, Van Raemdonck DE. Is it bronchiolitis obliterans syndrome or is it chronic rejection: a reappraisal? Eur Respir J. 2005;25:221–4.PubMedCrossRefGoogle Scholar
  25. 25.
    Verlden GM, Bankier A, Boehler A. Bronchiolitis obliterans syndrome after lung transplantation: diagnosis and treatment. Eur Respir Mon. 2004;29:1–25.CrossRefGoogle Scholar
  26. 26.
    Navarro J, Rainisio M, Harms HK, et al. Factors associated with poor pulmonary function: cross-sectional analysis of data from the ERCF. Eur Respir J. 2001;18:298–305.PubMedCrossRefGoogle Scholar
  27. 27.
    Scott RB, O’Loughlin EV, Gall DG. Gastroesophageal reflux in patients with cystic fibrosis. J Pediatr. 1985;106:223–7.PubMedCrossRefGoogle Scholar
  28. 28.
    Winnans CS, Harris LD. Quantitation of lower esophageal sphincter competence. Gastroenterology. 1967;52:773–8.Google Scholar
  29. 29.
    Woodley FW, Hayes J, Mousa H. Acid gastroesophageal reflux in symptomatic infants is primarily a function of classic 2-phase and pH-only acid reflux event types. J Pediatr Gastroenterol Nutr. 2009;48:550–8.PubMedCrossRefGoogle Scholar
  30. 30.
    Woodley FW, Mousa H. “pH-Only” acid reflux events in infants during later phases of the feeding cycle are less acidic and cleared more efficiently than classic 2-phase acid reflux events. J Pediatr Gastroenterol Nutr. 2009;48:41–7.PubMedCrossRefGoogle Scholar
  31. 31.
    Woodley FW, Machado R, Kaul A, et al. Using combined multichannel intraluminal impedance and esophageal pH monitoring to assess gastroesophageal reflux in children with cystic fibrosis. Manuscript in preparation. 2012.Google Scholar
  32. 32.
    Van der Lef HPJ, Arets HGM, Froeling SP. Gastric acid inhibition for fat malabsorption or gastroesophageal reflux disease in cystic fibrosis: Longitudinal effect on bacterial colonization and pulmonary function. J Pediatr. 2009;155:629–33.CrossRefGoogle Scholar
  33. 33.
    Nussbaum E, Maggi JC, Mathis R, et al. Association of lipid-laden alveolar macrophages and gastroesophageal reflux in children. J Pediatr. 1987;110:190–4.PubMedCrossRefGoogle Scholar
  34. 34.
    Kajetanowicz A, Stinson D, Laybolt KS, et al. Lipid-laden macrophages in the tracheal aspirate of ventilated neonates receiving Intralipid: a pilot study. Pediatr Pulmonol. 1999;28(2):101–8.PubMedCrossRefGoogle Scholar
  35. 35.
    Prakash UB, Rosenow EC. Pulmonary complications from ophthalmic preparations. Mayo Clin Proc. 1990;65(4):521–9.PubMedGoogle Scholar
  36. 36.
    Ramsey BW, Farrell PM, Pencharz P. Nutritional assessment and management in cystic fibrosis: a consensus report. Am J Clin Nutr. 1992;55:108–16.PubMedGoogle Scholar
  37. 37.
    Button BM, Heine RG, Catto-Smith AG. Postural drainage in cystic fibrosis: is there a link with gastro-oesophageal reflux? J Paediatr Child Health. 1998;34:330–4.PubMedCrossRefGoogle Scholar
  38. 38.
    Doumit M, Krishman U, Jaffe A, et al. Acid and non-acid reflux during physiotherapy in young children with cystic fibrosis. Pediatr Pumonol. 2012;47:119–24.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Center for Advanced Research in Neuromuscular Gastrointestinal DisordersThe Ohio State University, Nationwide Children’s HospitalColumbusUSA
  2. 2.Fellowship in the Advanced Training in Gastrointestinal Motility and Functional DisordersThe Ohio State University, Nationwide Children’s HospitalColumbusUSA
  3. 3.Division of Pediatric GastroenterologyThe Ohio State University College of Medicine, Nationwide Children’s HospitalColumbusUSA

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