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Gastroesophageal Reflux and Cystic Fibrosis

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Gastroesophageal Reflux in Children

Abstract

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.

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Abbreviations

BMI:

Body mass index

CF:

Cystic fibrosis

CFTR:

Cystic fibrosis transmembrane conductance regulator

FEV1:

Forced expiratory volume in 1 s

GER(D):

Gastroesophageal reflux (disease)

MII-pH:

Combined esophageal multichannel intraluminal impedance-pH monitoring

PPI(s):

Proton pump inhibitor(s)

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Authors and Affiliations

  1. Department of Gastroenterology, Hepatology and Nutrition, University Children’s Hospital Ljubljana, Bohoričeva ul. 20, SI-1000, Ljubljana, Slovenia

    Jernej Brecelj

  2. Department of Pediatrics, Faculty of Medicine, University of Ljubljana, Bohoričeva ul. 20, SI-1000, Ljubljana, Slovenia

    Jernej Brecelj

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Correspondence to Jernej Brecelj .

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  1. Universitair Ziekenhuis Brussel, Kidz Health Castle, Brussels, Belgium

    Yvan Vandenplas

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Brecelj, J. (2017). Gastroesophageal Reflux and Cystic Fibrosis. In: Vandenplas, Y. (eds) Gastroesophageal Reflux in Children. Springer, Cham. https://doi.org/10.1007/978-3-319-60678-1_15

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