GASTROENTEROLOGICAL ASPECTS OF CYSTIC-FIBROSIS

Cystic fibrosis (CF) is the most common hereditary disease amongst Caucasians with a potentially lethal outcome. The basic defect is a dysregulation of the chloride channels leading to a relative dehydration of the luminal surface of the exocrine cells. The prognosis of CF patients is predominantly related to the progression of their pulmonary disease. This prognosis has improved dramatically during the last decennia and many patients live into adulthood. Gastrointestinal disturbances and, consequently, malabsorption of nutrients represent a major therapeutic problem in CF. A better understanding of the nutritional problems, including the treatment of pancreatic insufficiency, appears to contribute to an improved prognosis. However, the nutritional status of many patients is still poor and studies to optimise treatment are of paramount importance. In addition, with the increased life span, hepatobiliary complications will contribute to the morbidity of these patients and preventive treatment may be warranted. The aim of this Symposium was to improve our knowledge in this important field by considering the interesting and up-to-date contributions of many experts.