pancreatic and biliary secretion are both altered in cystic fibrosis pigs

摘要

Liver involvement is also common in CF. Most patients with CF develop some evidence of liver abnormalities, including slightly elevated liver enzymes, fatty infiltration, and focal biliary cirrhosis (31, 86). Approximately 2-5% of patients develop advanced liver disease and liver failure (17). The gallbladder is also involved, and microgallbladder occurs in 20-30% of patients with CF (74, 85). Less than 10% of patients develop cholelithiasis/cholecystitis (80, 87).It has been difficult to separately study the function of the liver and pancreas in humans with CF. Pancreatic function studies in humans have been done by sampling the fluid in the jejunum (50, 51). Because the pancreatic duct converges with the common bile duct (CBD) shortly before opening at the duodenum, the jejunal fluid invariably contains a mixture of bile and pancreatic fluids. Furthermore, pancreatic duct and cholangiocyte epithelia secrete HCO_3~--rich fluid in response to secretin (43, 54, 55, 83); therefore, it is not possible to evaluate the relative contributions of biliary and pancreatic systems, unless the fluids are collected separately.