Beyond pancreatic insufficiency and liver disease in cystic fibrosis

Cystic fibrosis is a life shortening hereditary disease, primarily leading to progressive pulmonary infection and exocrine pancreatic dysfunction. Several gastrointestinal complications other than malabsorption can arise during the disease course and with the progressively increasing life span of patients with CF; new and more rare complications are being recognized. We review the literature on gastrointestinal manifestations in CF, excluding the liver and pancreas. Conclusion: We describe the clinical presentation and treatment of more common conditions like gastroesophageal reflux, small intestinal bacterial overgrowth, intussusception, meconium ileus, distal intestinal obstruction syndrome, and constipation, and we also discuss what is known on celiac disease, appendicitis, fibrosing colonopathy, inflammation and inflammatory bowel disease and gastrointestinal cancer.