Pancreatic insufficiency in CF2 of 2
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Absence or dysfunction of CFTR in pancreatic duct cells results in exocrine pancreatic insufficiency and consequential malnutrition.3, 4
In CF, CFTR dysfunction impairs or stops the secretion of Cl– and HCO3– into the lumen. Water secretion is also impaired as there is no longer a sufficient osmotic gradient to ‘drive’ water into the duct.1
This results in obstruction of ducts by a viscous secretion that prevents pancreatic enzymes from reaching the gut and functioning at an optimal pH.2
Over time, this can cause autolysis and inflammation of the exocrine pancreas. If left untreated, this leads to nutrient malabsorption, malnutrition and failure to thrive. As a consequence, many sufferers with CF are reliant on enzyme replacement therapy.