Gastroesophageal Reflux Disease and Need for Antireflux Surgery in Children with Cystic Fibrosis: A Systematic Review on Incidence, Surgical Complications, and Postoperative Outcomes

Ng, Jessica; Friedmacher, Florian; Pao, C.; Charlesworth, Paul

doi:10.1055/s-0040-1718750

Cited by 9 publications

(8 citation statements)

References 21 publications

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“…CF patients are at increased risk of exposure to the different antibiotics in CF patients that could alter the microbiome in the GI tract of those patients, making them more prone to different mucosal diseases including EoE [ 12 13 14 ]. CF patients also tend to have more GERD than the general population [ 29 30 31 ]. This could result in an increase in the use of PPIs which can be associated with an increase in EoE as reported previously [ 32 ].…”

Section: Discussionmentioning

confidence: 99%

Epidemiology of Eosinophilic Esophagitis in Patients with Cystic Fibrosis: A Population-Based 5-Year Study

Alaber

Sabe

Baez-Socorro

et al. 2022

Pediatr Gastroenterol Hepatol Nutr

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Purpose The prevalence of eosinophilic esophagitis (EoE) has been on the rise since it was first described in the 1990s. Several diseases and exogenous factors have been associated with EoE. Our aim was to investigate the epidemiology of EoE in cystic fibrosis (CF) patients. Methods We identified individuals with CF from September 2014 to September 2019 within a database (IBM Explorys Solutions, Inc.). The prevalence of EoE in patients with CF was compared to the general population. Results The database included 36,111,860 patients during the 5-year study period: 12,950 with CF (0.036%) and 28,090 with EoE (0.078%). EoE prevalence was higher in CF patients than the general population (46 in 10,000 vs. 7.8 in 10,000, p <0.001). Patients with CF and EoE were more likely to be male (50% vs. 33.5%, p <0.008), children (33.3% vs. 16.5%, p <0.001), and non-Hispanic (100% vs. 88.7%, p <0.001) than CF patients without EoE. CF with EoE patients were more likely to be children than EoE only (33.3% vs. 10.5%, p <0.001). Allergic conditions were generally more prevalent in CF with EoE than CF only (83.3% vs. 68.3%, p =0.01) and EoE only (83.3% vs. 69.3%, p =0.014). Conclusion EoE is nearly 6-times more prevalent in CF patients. Those patients had higher incidence of other atopic conditions. EoE must be considered in the differential diagnosis of patients with CF presenting with dysphagia, refractory gastroesophageal reflux, vomiting, and other esophagus-related symptoms.

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Section: Discussionmentioning

confidence: 99%

Epidemiology of Eosinophilic Esophagitis in Patients with Cystic Fibrosis: A Population-Based 5-Year Study

Alaber

Sabe

Baez-Socorro

et al. 2022

Pediatr Gastroenterol Hepatol Nutr

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“…A systematic review on incidence, surgical complications, and postoperative outcomes evaluated the results of antireflux surgery, including the Nissen fundoplication, for infants and children with CF 21 . This study showed that antireflux surgery improved GERD symptoms with a potential reduction in lung function decline.…”

Section: Pathophysiology Of the Cf Gi Tractmentioning

confidence: 96%

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

McDonald,

Reid,

Pohl

et al. 2024

Nut in Clin Prac

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Cystic fibrosis (CF) is a progressive, genetic, multi‐organ disease affecting the respiratory, digestive, endocrine, and reproductive systems. CF can affect any aspect of the gastrointestinal (GI) tract, including the esophagus, stomach, small intestine, colon, pancreas, liver, and gall bladder. GI pathophysiology associated with CF results from CF membrane conductance regulator (CFTR) dysfunction. The majority of people with CF (pwCF) experience exocrine pancreatic insufficiency resulting in malabsorption of nutrients and malnutrition. Additionally, other factors can cause or worsen fat malabsorption, including the potential for short gut syndrome with a history of meconium ileus, hepatobiliary diseases, and disrupted intraluminal factors, such as inadequate bile salts, abnormal pH, intestinal microbiome changes, and small intestinal bacterial overgrowth. Signs and symptoms associated with fat malabsorption, such as abdominal pain, bloating, malodorous flatus, gastroesophageal reflux, nausea, anorexia, steatorrhea, constipation, and distal intestinal obstruction syndrome, are seen in pwCF despite the use of pancreatic enzyme replacement therapy. Given the association of poor nutrition status with lung function decline and increased mortality, aggressive nutrition support is essential in CF care to optimize growth in children and to achieve and maintain a healthy body mass index in adults. The introduction of highly effective CFTR modulator therapy and other advances in CF care have profoundly changed the course of CF management. However, GI symptoms in some pwCF may persist. The use of current knowledge of the pathophysiology of the CF GI tract as well as appropriate, individualized management of GI symptoms continue to be integral components of care for pwCF.

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“…Basic nutritional care includes a high-calorie, high-fat diet associated with PERT, and fat-soluble vitamins supplementation. Morbidities like pulmonary infections, gastrointestinal complication (gastro-esophageal reflux, constipation, distal intestinal obstructive syndrome) can decrease appetite and determine inadequate nutrient intake [ 31 , 32 , 33 ].…”

Section: Clinical Considerations Of Exocrine Pancreatic Insufficiencymentioning

confidence: 99%

Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine

et al. 2023

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This review presents current updates of pancreatic enzyme replacement therapy in children with cystic fibrosis based on literature published in the last decade and some special considerations regarding pancreatic enzyme replacement therapy in the era of new therapies, such as cystic fibrosis transmembrane conductance regulator modulator therapies. Few articles evaluate the efficacy of pancreatic enzyme replacement therapy in the pediatric population, and most studies also included children and adults with cystic fibrosis. Approximately 85% of cystic fibrosis patients have exocrine pancreatic insufficiency and need pancreatic enzyme replacement therapy. Fecal elastase is the most commonly used diagnostic test for exocrine pancreatic insufficiency, although this value can fluctuate over time. While it is used as a diagnostic test, it cannot be used for monitoring the effectiveness of pancreatic enzyme replacement therapy and for adjusting doses. Pancreatic enzyme replacement therapy, the actual treatment for exocrine pancreatic insufficiency, is essential in children with cystic fibrosis to prevent malabsorption and malnutrition and needs to be urgently initiated. This therapy presents many considerations for physicians, patients, and their families, including types and timing of administration, dose monitoring, and therapy failures. Based on clinical trials, pancreatic enzyme replacement therapy is considered effective and well-tolerated in children with cystic fibrosis. An important key point in cystic fibrosis treatment is the recent hypothesis that cystic fibrosis transmembrane conductance regulator modulators could improve pancreatic function, further studies being essential. Pancreatic enzyme replacement therapy is addressed a complication of the disease (exocrine pancreatic insufficiency), while modulators target the defective cystic fibrosis transmembrane conductance regulator protein. Exocrine pancreatic insufficiency in cystic fibrosis remains an active area of research in this era of cystic fibrosis transmembrane conductance regulator modulator therapies. This new therapy could represent an example of personalized medicine in cystic fibrosis patients, with each class of modulators being addressed to patients with specific genetic mutations.

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Gastroesophageal Reflux Disease and Need for Antireflux Surgery in Children with Cystic Fibrosis: A Systematic Review on Incidence, Surgical Complications, and Postoperative Outcomes

Cited by 9 publications

References 21 publications

Epidemiology of Eosinophilic Esophagitis in Patients with Cystic Fibrosis: A Population-Based 5-Year Study

Epidemiology of Eosinophilic Esophagitis in Patients with Cystic Fibrosis: A Population-Based 5-Year Study

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine

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