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World J Gastroenterol. Nov 7, 2020; 26(41): 6322-6334
Published online Nov 7, 2020. doi: 10.3748/wjg.v26.i41.6322
Focus on gastroesophageal reflux disease in patients with cystic fibrosis
Annarita Bongiovanni, Sara Manti, Giuseppe Fabio Parisi, Maria Papale, Enza Mulè, Novella Rotolo, Salvatore Leonardi
Annarita Bongiovanni, Sara Manti, Giuseppe Fabio Parisi, Maria Papale, Enza Mulè, Novella Rotolo, Salvatore Leonardi, Department of Clinical and Experimental Medicine, Pediatric Respiratory Unit, San Marco Hospital, University of Catania, Catania 95123, Italy
Author contributions: Bongiovanni A and Manti S contributed equally to this work; Parisi GF reviewed the paper; Papale M and Mulè E performed the research; Rotolo N analyzed the data; Leonardi S supervised the work; all authors have read and approve the final manuscript.
Conflict-of-interest statement: The authors have no conflicts of interest to disclose that could be perceived as prejudicing the impartiality of the research reported.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Sara Manti, MD, PhD, Postdoc, Department of Clinical and Experimental Medicine, University of Catania, Via Santa Sofia, 78, Catania 95123, Italy. saramanti@hotmail.it
Received: June 18, 2020
Peer-review started: June 18, 2020
First decision: July 28, 2020
Revised: August 22, 2020
Accepted: October 1, 2020
Article in press: October 1, 2020
Published online: November 7, 2020
Processing time: 141 Days and 2.2 Hours
Abstract

Gastroesophageal reflux disease (GERD) is a common gastrointestinal disorder in cystic fibrosis (CF), and based on various studies, its prevalence is elevated since childhood. There are several pathogenetic mechanisms on the basis of association between CF and GERD. However, there are no specific guidelines for GERD in CF patients, so diagnosis is based on guidelines performed on patients not affected by CF. The aim of this review is to provide the pathophysiology, diagnostic and therapeutic options, complications, and future directions in the management of GERD patients with CF.

Keywords: Adult; Children; Cystic Fibrosis; Gastroesophageal reflux

Core Tip: Gastroesophageal reflux (GER) is common in patients with cystic fibrosis (CF) and is often regarded as playing a role in the pathogenesis of CF lung disease. Several studies have showed that patients with CF who have coexisting GER have more severe pulmonary disease with an increased numbers of infectious exacerbations as well as a lower pulmonary function. An adequate diagnostic and therapeutic management can significantly improve respiratory morbidity. Because of the lack of specific guidelines for management of GER in CF, currently, diagnosis and treatment for symptomatic GER in CF patients is based on standard guidelines for the general population. Herein, we aimed to provide a pragmatic update on the pathophysiology, diagnostic work-up, and therapeutic options in the GERD patients with CF.