Cystic Fibrosis and Celiac Disease

Pohl, John F.; Lowichik, Amy; Cantrell, Amy

doi:10.1177/0009922811388512

Cited by 7 publications

(1 citation statement)

References 10 publications

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“…Interestingly, cystic fibrosis transmembrane conductance regulator (CFTR) modulators used in CF also have been shown to alter intestinal microbiota and have been associated with celiac disease (CD) in small studies [7][8][9][10][11]. Small case series of pediatric patients with CF have described associated CD, eosinophilic esophagitis (EoE), and inflammatory bowel disease [11][12][13][14][15][16]. Furthermore, studies including both adults and children with CF have demonstrated a potential increased incidence of CD and EoE [5,17].…”

Section: Introductionmentioning

confidence: 99%

Gastrointestinal Pathologies in Pediatric Patients With Cystic Fibrosis Undergoing Endoscopy: A Single-Center Retrospective Review Over 15 Years

Blaseg,

Robson,

Patel

et al. 2024

Cureus

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IntroductionPrevious studies have demonstrated an increased incidence of gastrointestinal (GI) pathologies, specifically celiac disease (CD) and eosinophilic esophagitis (EoE), in patients with cystic fibrosis (CF). However, there is minimal data available regarding endoscopic findings in pediatric patients with CF and GI mucosal disease. MethodsA retrospective chart review was performed on patients with CF under 18 years of age who underwent esophagogastroduodenoscopy (EGD) or colonoscopy with biopsy over a 15-year period at our institution. Patient characteristics including assigned sex at birth, CF genetic mutations (if identified), and cystic fibrosis transmembrane conductance regulator (CFTR) modulator use were recorded. Data obtained at the time of biopsy included body mass index (BMI), indication for the procedure, exocrine pancreatic status, visual endoscopic findings, and histologic findings. ResultsA total of 72 patients with CF were included in the study. 24% (n=17) were found to have abnormal endoscopic biopsy results. EoE (13% of all patients, n=9) and CD (6% of all patients, n=4) were the most common GI diagnoses present on endoscopic biopsy. All 3 patients taking CFTR modulator medications at the time of endoscopy had normal biopsy results. Of the 17 patients found to have abnormal pathology results, 14 (82%) were taking proton-pump inhibitor (PPI) medication at the time of endoscopy. ConclusionThis study highlights the probable increased frequency of GI disease in the pediatric CF population. These findings underscore the importance of maintaining a broad differential diagnosis while considering utilization of endoscopy with biopsy in pediatric patients with CF who have GI symptoms.

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Section: Introductionmentioning

confidence: 99%

Gastrointestinal Pathologies in Pediatric Patients With Cystic Fibrosis Undergoing Endoscopy: A Single-Center Retrospective Review Over 15 Years

Blaseg,

Robson,

Patel

et al. 2024

Cureus

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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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Nutrition for Pancreatic Sufficient Individuals with Cystic Fibrosis

Pohl

McDonald

2015

Nutrition in Cystic Fibrosis

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Cystic Fibrosis and Celiac Disease

Cited by 7 publications

References 10 publications

Gastrointestinal Pathologies in Pediatric Patients With Cystic Fibrosis Undergoing Endoscopy: A Single-Center Retrospective Review Over 15 Years

Gastrointestinal Pathologies in Pediatric Patients With Cystic Fibrosis Undergoing Endoscopy: A Single-Center Retrospective Review Over 15 Years

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

Nutrition for Pancreatic Sufficient Individuals with Cystic Fibrosis

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