Cystic fibrosis overview of gastrointestinal disease gas finder near me


Cystic fibrosis (CF) generally is thought of as a lung disease since much of the associated morbidity and mortality is related to pulmonary complications. A discussion of the pulmonary manifestations of CF is presented separately. (See "Cystic fibrosis: Clinical manifestations of pulmonary disease" and "Cystic fibrosis: Overview of the treatment of lung disease".)

The underlying pathophysiology of CF is related to abnormal chloride and bicarbonate transport caused by mutations in the CF transmembrane conductance regulator gene (CFTR) located on chromosome 7. The mutations cause the production of abnormally tenacious mucus and secretions in the lungs, gastrointestinal tract, pancreas, and hepatobiliary system. As a result, the lumens of these organs become obstructed leading to the clinical findings associated with this disease process. (See "Cystic fibrosis: Genetics and pathogenesis".)

Gastrointestinal complications have become an increasingly important cause of morbidity in patients with CF, in part because of improved life expectancy. Some of the complications, such as gastroesophageal reflux and constipation, are not unique to the CF population, but they are more common and warrant special considerations in diagnosis and management in the CF population. This topic review provides an overview of the gastrointestinal manifestations of CF. Pancreatic insufficiency, which is one of the most clinically important gastrointestinal issues, and other nutritional issues are addressed separately. (See "Cystic fibrosis: Assessment and management of pancreatic insufficiency" and "Cystic fibrosis: Nutritional issues".)

Gastroesophageal reflux disease (GERD) — Gastroesophageal reflux disease (GERD) appears to be more common in individuals with CF as compared with the general population [ 1,2]. Symptomatic GERD is reported in 30 to 40 percent of individuals with CF, and "silent" GERD detected by esophageal monitoring has been reported in up to 90 percent of individuals with severe pulmonary disease [ 3]. However, the magnitude of the association has not been well-defined, since GERD is common in otherwise healthy individuals and because CF is associated with other gastrointestinal disorders that may contribute to or be confused with GERD ( table 1). (See "Clinical manifestations and diagnosis of gastroesophageal reflux disease in children and adolescents".)

The principal mechanism causing GERD in children with CF appears to be transient relaxation of the lower esophageal sphincter (LES), which is also the most common mechanism leading to GERD in patients without CF. Several other mechanisms may also contribute to GERD in individuals with CF:

• van der Doef HP, Arets HG, Froeling SP, et al. Gastric acid inhibition for fat malabsorption or gastroesophageal reflux disease in cystic fibrosis: longitudinal effect on bacterial colonization and pulmonary function. J Pediatr 2009; 155:629.

• Abbassi-Ghadi N, Kumar S, Cheung B, et al. Anti-reflux surgery for lung transplant recipients in the presence of impedance-detected duodenogastroesophageal reflux and bronchiolitis obliterans syndrome: a study of efficacy and safety. J Heart Lung Transplant 2013; 32:588.

• Kopelman H, Forstner G, Durie P, Corey M. Origins of chloride and bicarbonate secretory defects in the cystic fibrosis pancreas, as suggested by pancreatic function studies on control and CF subjects with preserved pancreatic function. Clin Invest Med 1989; 12:207.

• Duytschaever G, Huys G, Bekaert M, et al. Cross-sectional and longitudinal comparisons of the predominant fecal microbiota compositions of a group of pediatric patients with cystic fibrosis and their healthy siblings. Appl Environ Microbiol 2011; 77:8015.

• del Campo R, Garriga M, Pérez-Aragón A, et al. Improvement of digestive health and reduction in proteobacterial populations in the gut microbiota of cystic fibrosis patients using a Lactobacillus reuteri probiotic preparation: a double blind prospective study. J Cyst Fibros 2014; 13:716.

• Moran A, Brunzell C, Cohen RC, et al. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care 2010; 33:2697.