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

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 Evaluation of Quality of Life in Terms of Sinonasal Symptoms in Children with Cystic Fibrosis

2019 ◽  
Vol 10 (1) ◽  
pp. 91-98 ◽  
Author(s):  
Maryam Hassanzad ◽  
Kourosh Fakhimi Derakhshan ◽  
Hosseinali Ghaffaripour ◽  
Ali Safavi Naeini ◽  
Habib Emami ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Chronic Rhinosinusitis ◽  
Nasal Obstruction ◽  
Diagnostic Study ◽  
Sinus Infection ◽  
Postnasal Drip ◽  
Significant Difference ◽  
The Mean

AbstractObjectiveSinusitis is a common complaint in children with cystic fibrosis. However, the actual prevalence of chronic rhinosinusitis and its effect on the quality of life of children have not been well considered. Therefore, the objective of this study was to determine the effect of sinonasal quality of life in children with cystic fibrosis.Materials and methodsThis study was a diagnostic study performed on 80 children with cystic fibrosis ranging from 2 to 20 years old, who were referred to the cystic fibrosis clinic of Masih Daneshvari Hospital from 2017–2018. The questionnaires used in this study were chronic rhinosinusitis screening questionnaire based on the European task force and the evaluation of the sinonasal quality of life was based on the SN-5 survey.ResultsOf the 80 patients with fibrosis from 2 to 20 years old who were recruited in the study, 41 patients were female (51.3%) and 39 were male (48.8%). In 61 cases (76.3%), there was no chronic rhinosinusitis and 19 cases (23.8%) had chronic rhinosinusitis. The mean SN-5 score in 19 patients with chronic rhinosinusitis was 3.4105 and the mean score of patients without rhinosinusitis was 1.8426, with a P-value of 0.000. The mean SN-5 score was significant between the two groups. In patients with nasal congestion, there was a significant difference in quality of life factors such as sinus infection, nasal obstruction, and allergy symptoms (P<0.001). In patients with facial pain, there was a significant difference in quality of life factors such as sinus infection, nasal obstruction, allergic symptoms, and physical activity limitation (P <0.001). There was also a significant difference in the quality of life factors such as sinus infection, nasal obstruction, and allergy symptoms in patients with postnasal drip (P <0.001).ConclusionIn children with cystic fibrosis, the quality of life of sinonasal has a significant relationship with absence of chronic rhinosinusitis. This study showed that children with chronic renosinusitis have significantly lower quality of sinonasal life than children with chronic rhinosinusitis. The results demonstrated that quality of life scores in sinus infections, nasal obstruction, and allergy symptoms were significantly higher in patients with chronic rhinosinusitis than in those without chronic rhinosinusitis. The findings of this study are important for improving children’s health related quality of life, as it leads to promoting communication between the patient and the health care provider, identifying overlooked problems, monitoring the progress of the disease and the burden of treatment, and promoting interventions in the daily life of patients.

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 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
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