scholarly journals Early intrahepatic duct defects in a cystic fibrosis porcine model

2021 ◽  
Vol 9 (14) ◽  
Author(s):  
Keyan Zarei ◽  
David K. Meyerholz ◽  
David A. Stoltz
Keyword(s):  
Cystic Fibrosis ◽  
Porcine Model ◽  
Intrahepatic Duct

scholarly journals Pathophysiologic evaluation of the transgenic CFTR “gut-corrected” porcine model of cystic fibrosis

2016 ◽  
Vol 311 (4) ◽  
pp. L779-L787 ◽  
Author(s):  
Stephen T. Ballard ◽  
Jessica W. Evans ◽  
Holly S. Drag ◽  
Michele Schuler
Keyword(s):  
Cystic Fibrosis ◽  
Plasma Proteins ◽  
Porcine Model ◽  
Liver Enzymes ◽  
Neonatal Period ◽  
Genetic Manipulation ◽  
Mucociliary Transport ◽  
Metabolic Dysfunction ◽  
Plasma Analysis ◽  
Elevated Liver Enzymes

This study evaluated the pulmonary pathophysiology of pigs with transgenic CFTR “gut-corrected” cystic fibrosis (CF). Four sows produced 18 piglets of which 11 were stillborn, with only 2 animals surviving beyond 2 wk. Failure to survive beyond the neonatal period by five piglets was judged to result from metabolic dysfunction related to genetic manipulation for CFTR gut expression or due to cloning artifact. Plasma analysis showed very low plasma proteins, highly elevated liver enzymes, and severe acidosis. All surviving offspring received furosemide for systemic edema. Physiologic evaluation was performed with lung tissues from the two surviving pigs. Both acetylcholine and forskolin induced mucous liquid secretion that was significantly lower in bronchi of pigs with CF than those without CF. The percent of nonvolatile solids in mucus secreted from CF bronchi was elevated following acetylcholine or forskolin treatment. Mucociliary transport in excised tracheas was reduced in the CF tracheas relative to non-CF tracheas. The diameter of tracheas in pigs with CF was less than that of pigs without CF despite their greater body weight. Despite exhibiting severe metabolic dysfunction during the neonatal period, this CF animal model appears to express important characteristics of human CF pulmonary disease.

Transepithelial Electrolyte And Fluid Transport In Type II Alveolar Epithelia In A Porcine Model Of Cystic Fibrosis

2010 ◽  
Author(s):  
Xiaopeng Li ◽  
Philip Karp ◽  
Peter Taft ◽  
Alejandro Pezzulo ◽  
David Stoltz ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Fluid Transport ◽  
Porcine Model ◽  
Type Ii

scholarly journals Early pathogenesis of cystic fibrosis gallbladder disease in a porcine model

2020 ◽  
Vol 100 (11) ◽  
pp. 1388-1399
Author(s):  
Keyan Zarei ◽  
Mallory R. Stroik ◽  
Nick D. Gansemer ◽  
Andrew L. Thurman ◽  
Lynda S. Ostedgaard ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Porcine Model ◽  
Gallbladder Disease

scholarly journals Pathology of Gastrointestinal Organs in a Porcine Model of Cystic Fibrosis

2010 ◽  
Vol 176 (3) ◽  
pp. 1377-1389 ◽  
Author(s):  
David K. Meyerholz ◽  
David A. Stoltz ◽  
Alejandro A. Pezzulo ◽  
Michael J. Welsh
Keyword(s):  
Cystic Fibrosis ◽  
Porcine Model

scholarly journals Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis

2012 ◽  
Vol 122 (9) ◽  
pp. 1898-1905 ◽  
Author(s):  
Eugene H. Chang ◽  
Alejandro A. Pezzulo ◽  
David K. Meyerholz ◽  
Andrea E. Potash ◽  
Tanner J. Wallen ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Porcine Model ◽  
Sinus Infection
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