The pancreas, liver and gallbladder are commonly involved in cystic fibrosis (CF) and the presence of acidic, dehydrated, and protein-rich secretions are characteristic findings. Pancreatic function studies in humans have been done by sampling the jejunal fluid. However, it has been difficult to separately study the function of pancreatic and biliary systems in humans with CF, because jejunal fluid contains a mixture of bile and pancreatic fluids. In contrast, pancreatic and biliary ducts open separately into the porcine intestine, therefore biliary and pancreatic fluid can be individually analyzed in CF pigs. We studied newborn wild-type (WT) and CF pigs and found that CFTR was localized to the pancreatic ducts. We collected bile and pancreatic fluid and analyzed pancreatic enzymes with activity assays and immunoblot. Pancreatic enzyme expression was significantly decreased in CF pigs compared to WT. The volume and pH of pancreatic fluid were significantly lower and protein concentration was >5-fold higher in CF. Secretin stimulation increased pancreatic fluid volume and pH in WT pigs, but not in CF. Baseline bile volume did not differ between WT and CF pigs, but volume did not increase in response to secretin in CF. The bile pH was lower and protein concentration was 2-fold higher in CF. These results indicate that both pancreatic and biliary secretions are altered in CF pigs. Abnormal pancreatic and biliary secretion in CF may have important implications in disease pathogenesis.
- Copyright © 2012, American Journal of Physiology- Gastrointestinal and Liver Physiology