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

Overview

Journal Curr Gastroenterol Rep

Specialty Gastroenterology

Date 2012 Apr 25

PMID 22528661

Citations 12

Authors

Hayat M Mousa

Frederick W Woodley

Affiliations

Soon will be listed here.

Abstract

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.

Citing Articles

The current state of gastrointestinal motility evaluation in cystic fibrosis: a comprehensive literature review.

Davis T, Miller A, Hachem C, Velez C, Patel D Transl Gastroenterol Hepatol. 2024; 9:10.

PMID: 38317748 PMC: 10838618. DOI: 10.21037/tgh-23-59.

A Gastroenterologist's Guide to Care Transitions in Cystic Fibrosis from Pediatrics to Adult Care.

Patel D, Baliss M, Saikumar P, Numan L, Teckman J, Hachem C Int J Mol Sci. 2023; 24(21).

PMID: 37958749 PMC: 10648514. DOI: 10.3390/ijms242115766.

Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management.

Patel D, Shan A, Mathews S, Sathe M Nutrients. 2022; 14(5).

PMID: 35268004 PMC: 8912424. DOI: 10.3390/nu14051028.

Systems Biology and Bile Acid Signalling in Microbiome-Host Interactions in the Cystic Fibrosis Lung.

Woods D, Flynn S, Caparros-Martin J, Stick S, Reen F, OGara F Antibiotics (Basel). 2021; 10(7).

PMID: 34202495 PMC: 8300688. DOI: 10.3390/antibiotics10070766.

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents.

Dos Santos A, de Melo Santos H, Nogueira M, Tavora H, de Lourdes Jaborandy Paim da Cunha M, Seixas R Pediatr Gastroenterol Hepatol Nutr. 2018; 21(4):306-314.

PMID: 30345244 PMC: 6182489. DOI: 10.5223/pghn.2018.21.4.306.

References

Fathi H, Moon T, Donaldson J, Jackson W, Sedman P, Morice A . Cough in adult cystic fibrosis: diagnosis and response to fundoplication. Cough. 2009; 5:1. PMC: 2634760. DOI: 10.1186/1745-9974-5-1. View

Bontempo I, Piretta L, Corazziari E, Michetti F, Anzini F, TORSOLI A . Effects of intraluminal acidification on oesophageal motor activity. Gut. 1994; 35(7):884-90. PMC: 1374833. DOI: 10.1136/gut.35.7.884. View

Ramsey B, Farrell P, Pencharz P . Nutritional assessment and management in cystic fibrosis: a consensus report. The Consensus Committee. Am J Clin Nutr. 1992; 55(1):108-16. DOI: 10.1093/ajcn/55.1.108. View

Cucchiara S, Raia V, Minella R, Frezza T, De Vizia B, De Ritis G . Ultrasound measurement of gastric emptying time in patients with cystic fibrosis and effect of ranitidine on delayed gastric emptying. J Pediatr. 1996; 128(4):485-8. DOI: 10.1016/s0022-3476(96)70358-x. View

Gregory P . Gastrointestinal pH, motility/transit and permeability in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1996; 23(5):513-23. DOI: 10.1097/00005176-199612000-00001. View

Young L, Hadjiliadis D, Davis R, Palmer S . Lung transplantation exacerbates gastroesophageal reflux disease. Chest. 2003; 124(5):1689-93. DOI: 10.1378/chest.124.5.1689. View

Nussbaum E, Maggi J, Mathis R, Galant S . Association of lipid-laden alveolar macrophages and gastroesophageal reflux in children. J Pediatr. 1987; 110(2):190-4. DOI: 10.1016/s0022-3476(87)80152-x. View

Gustafsson P, Fransson S, Kjellman N, Tibbling L . Gastro-oesophageal reflux and severity of pulmonary disease in cystic fibrosis. Scand J Gastroenterol. 1991; 26(5):449-56. DOI: 10.3109/00365529108998565. View

Woodley F, 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(1):41-7. DOI: 10.1097/MPG.0b013e31816f214a. View

10.

Doumit M, Krishnan U, Jaffe A, Belessis Y . Acid and non-acid reflux during physiotherapy in young children with cystic fibrosis. Pediatr Pulmonol. 2012; 47(2):119-24. DOI: 10.1002/ppul.21524. View

11.

Woodley F, 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(5):550-8. DOI: 10.1097/MPG.0b013e318185dae7. View

12.

Dab I, Malfroot A . Gastroesophageal reflux: a primary defect in cystic fibrosis?. Scand J Gastroenterol Suppl. 1988; 143:125-31. DOI: 10.3109/00365528809090232. View

13.

Kajetanowicz A, Stinson D, Laybolt K, Resch L . Lipid-laden macrophages in the tracheal aspirate of ventilated neonates receiving Intralipid: A pilot study. Pediatr Pulmonol. 1999; 28(2):101-8. DOI: 10.1002/(sici)1099-0496(199908)28:2<101::aid-ppul5>3.0.co;2-g. View

14.

Malfroot A, Dab I . New insights on gastro-oesophageal reflux in cystic fibrosis by longitudinal follow up. Arch Dis Child. 1991; 66(11):1339-45. PMC: 1793275. DOI: 10.1136/adc.66.11.1339. View

15.

Prakash U, Rosenow 3rd E . Pulmonary complications from ophthalmic preparations. Mayo Clin Proc. 1990; 65(4):521-9. DOI: 10.1016/s0025-6196(12)60952-5. View

16.

Scott R, OLoughlin E, Gall D . Gastroesophageal reflux in patients with cystic fibrosis. J Pediatr. 1985; 106(2):223-7. DOI: 10.1016/s0022-3476(85)80291-2. View

17.

Blondeau K, Pauwels A, Dupont L, Mertens V, Proesmans M, Orel R . Characteristics of gastroesophageal reflux and potential risk of gastric content aspiration in children with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2009; 50(2):161-6. DOI: 10.1097/MPG.0b013e3181acae98. View

18.

van der Doef H, Arets H, Froeling S, Westers P, Houwen R . 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(5):629-33. DOI: 10.1016/j.jpeds.2009.06.040. View

19.

Button B, Roberts S, Kotsimbos T, Levvey B, Williams T, Bailey M . 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):1522-9. DOI: 10.1016/j.healun.2004.11.312. View

20.

Blondeau K, Mertens V, Vanaudenaerde B, Verleden G, Van Raemdonck D, Sifrim D . Gastro-oesophageal reflux and gastric aspiration in lung transplant patients with or without chronic rejection. Eur Respir J. 2007; 31(4):707-13. DOI: 10.1183/09031936.00064807. View