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About CF » Pathophysiology of CF » GastrointestinaI tract

GastrointestinaI tract



CFTR is present on the epithelial cells lining the gastrointestinal tract (GI). Defects in CFTR lead to reduced secretion of chloride and bicarbonate into the intestinal tract and hyperabsorption of water from the gut. This causes accumulation of viscous mucus and faecal material in the terminal ileum, caecum and ascending colon leading to obstruction by mucofaeculant material.1 In fact, up to 15% of  newborn babies with CF suffer from intestinal obstruction,2, 3 and up to 18% of adults suffer from a recurrent bowel obstruction known as distal intestinal obstructive syndrome (DIOS).4

Viscous mucus and faecal material often obstruct the GI in people with cystic fibrosis. This leads to nutrient malabsorption compounded by low duodenal pH, which is a consequence of pancreatic insufficiency
  1. Walker NM et al. J Pharmacol Exp Ther 2006;317:275–83
  2. Rowe SM & Clancy JP. Curr Opin Pediatr 2006;18:604–13
  3. Walkowiak J et al. Eur J Gastroenterol Hepatol 2008;20:157–60
  4. Dray X et al. Clin Gastroenterol Hepatol 2004;2:498–503