title: Pancreatic and biliary secretion are both altered in cystic fibrosis pigs.
abstract: The pancreas, liver, and gallbladder are commonly involved in cystic fibrosis (CF), and 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 compared with WT pigs. The volume and pH of pancreatic fluid were significantly lower and protein concentration was >5-fold higher in CF pigs. Secretin stimulation increased pancreatic fluid volume and pH in WT, but not CF, pigs. Baseline bile volume did not differ between WT and CF pigs, but volume did not increase in response to secretin in CF pigs. Bile pH was lower and protein concentration was twofold higher in CF pigs. These results indicate that pancreatic and biliary secretions are altered in CF pigs. Abnormal pancreatic and biliary secretion in CF may have important implications in disease pathogenesis.
authors.affiliation: Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, Iowa 52242, USA. aliye-uc@uiowa.edu
authors.forename: Aliye
authors.initials: A
authors.lastname: Uc
authors.forename: Radhamma
authors.initials: R
authors.lastname: Giriyappa
authors.forename: David K
authors.initials: DK
authors.lastname: Meyerholz
authors.forename: Michelle
authors.initials: M
authors.lastname: Griffin
authors.forename: Lynda S
authors.initials: LS
authors.lastname: Ostedgaard
authors.forename: Xiao Xiao
authors.initials: XX
authors.lastname: Tang
authors.forename: Marwa
authors.initials: M
authors.lastname: Abu-El-Haija
authors.forename: David A
authors.initials: DA
authors.lastname: Stoltz
authors.forename: Paula
authors.initials: P
authors.lastname: Ludwig
authors.forename: Alejandro
authors.initials: A
authors.lastname: Pezzulo
authors.forename: Maisam
authors.initials: M
authors.lastname: Abu-El-Haija
authors.forename: Peter
authors.initials: P
authors.lastname: Taft
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Welsh
mesh_terms: D000681:Amylases / Q000378:metabolism; D000818:Animals; D001652:Bile Ducts / Q000378:metabolism* / Q000503:physiopathology; D003550:Cystic Fibrosis / Q000378:metabolism* / Q000503:physiopathology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism; D004195:Disease Models, Animal; D010179:Pancreas / Q000378:metabolism* / Q000503:physiopathology; D010189:Pancreatic Juice / Q000378:metabolism; D013552:Swine; D014357:Trypsin / Q000378:metabolism
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D000681:Amylases; D014357:Trypsin