Gastroesophageal Reflux and Cystic Fibrosis

  • Jernej BreceljEmail author


Cystic fibrosis is an autosomal recessive genetic disease characterized by chronic suppurative lung disease, exocrine pancreatic dysfunction, hepatobiliary disease, gastrointestinal disease, and many other clinical manifestations. Gastroesophageal reflux is a primary phenomenon in cystic fibrosis patients and is more prevalent than in general population. Lung aspirations of duodenogastric fluid are an underestimated risk factor for the lung disease progression. Advanced lung disease additionally increases gastroesophageal reflux risk. Many symptoms and signs of cystic fibrosis are overlapping with those of gastroesophageal reflux disease and are not a prognostic factor for its presence or severity level. Despite a lot of evidence, controversies regarding gastroesophageal reflux disease diagnosis and treatment in cystic fibrosis patients still exist. One of diagnostic challenges is lung aspiration detection. Proton pump inhibitors are the mainstay of the treatment employed in half of all patients. Antireflux operation in selected patients probably slows the decline of lung function. In addition, other topics of interest in cystic fibrosis patients interrelated with gastroesophageal reflux are addressed: respiratory physiotherapy, gastrostomy, and lung transplantation. With the prolongation of life expectancy, late complications of gastroesophageal reflux disease will become more prevalent. Gastroesophageal reflux in cystic fibrosis patients is a challenging field for clinical practice and research.


Gastroesophageal reflux disease Cystic fibrosis Combined esophageal multichannel intraluminal impedance-pH monitoring Baseline impedance Gastric emptying Proton pump inhibitors Fundoplication Respiratory physiotherapy Gastrostomy Lung transplantation Barrett’s esophagus 



Body mass index


Cystic fibrosis


Cystic fibrosis transmembrane conductance regulator


Forced expiratory volume in 1 s


Gastroesophageal reflux (disease)


Combined esophageal multichannel intraluminal impedance-pH monitoring


Proton pump inhibitor(s)


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Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Department of Gastroenterology, Hepatology and NutritionUniversity Children’s Hospital LjubljanaLjubljanaSlovenia
  2. 2.Department of Pediatrics, Faculty of MedicineUniversity of LjubljanaLjubljanaSlovenia

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