Sinus Hypoplasia Precedes Sinus Infection in a Porcine Model of Cystic Fibrosis

Eugene H CHANG; Alejandro A PEZZULO; Nicholas D GANSEMER; Paul B MCCRAY; David A STOLTZ; Michael J WELSH; Joseph ZABNER; David K MEYERHOLZ; Andrea E POTASH; Tanner J WALLEN; Leah R REZNIKOV; Jessica C SIEREN; Philip H KARP; Sarah ERNST; Thomas O MONINGER

doi:10.1002/lary.23392

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Sinus Hypoplasia Precedes Sinus Infection in a Porcine Model of Cystic Fibrosis

Journal article

Open access

Peer reviewed

Sinus Hypoplasia Precedes Sinus Infection in a Porcine Model of Cystic Fibrosis

Eugene H CHANG, Alejandro A PEZZULO, Nicholas D GANSEMER, Paul B MCCRAY, David A STOLTZ, Michael J WELSH, Joseph ZABNER, David K MEYERHOLZ, Andrea E POTASH, Tanner J WALLEN, …

The Laryngoscope, Vol.122(9), pp.1898-1905

2012

DOI: 10.1002/lary.23392

PMCID: PMC3449319

PMID: 22711071

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Abstract

Objectives/Hypothesis: Chronic sinusitis is nearly universal in humans with cystic fibrosis (CF) and is accompanied by sinus hypoplasia (small sinuses). However, whether impaired sinus development is a primary feature of loss of the cystic fibrosis transmembrane conductance regulator (CFTR) or a secondary consequence of chronic infection remains unknown. Our objective was to study the early pathogenesis of sinus disease in CF. Study Design: Animal/basic science research. Methods: Sinus development was studied in a porcine CF model. Results: Porcine sinus epithelia expressed CFTR and exhibited transepithelial anion transport. Disruption of the CFTR gene eliminated both. Sinuses of newborn CF pigs were not infected and showed no evidence of inflammation, yet were hypoplastic at birth. Older CF pigs spontaneously developed sinus disease similar to that seen in humans with CF. Conclusions: These results define a role for CFTR in sinus development and suggest the potential of the CF pig as a genetic model of CF-sinus disease in which to test therapeutic strategies to minimize sinus-related CF morbidity.

Errors of metabolism

Biological and medical sciences

Metabolic diseases

Medical sciences

Otorhinolaryngology. Stomatology

Miscellaneous hereditary metabolic disorders

Details

Title: Subtitle: Sinus Hypoplasia Precedes Sinus Infection in a Porcine Model of Cystic Fibrosis
Creators: Eugene H CHANG - Department of Otolaryngology―Head and Neck Surgery, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Alejandro A PEZZULO - Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Nicholas D GANSEMER - Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Paul B MCCRAY - Department of Pediatrics, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
David A STOLTZ - Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Michael J WELSH - Howard Hughes Medical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Joseph ZABNER - Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
David K MEYERHOLZ - Department of Pathology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Andrea E POTASH - Department of Otolaryngology―Head and Neck Surgery, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Tanner J WALLEN - Department of Otolaryngology―Head and Neck Surgery, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Leah R REZNIKOV - Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Jessica C SIEREN - Department of Radiology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Philip H KARP - Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Sarah ERNST - Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Thomas O MONINGER - Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, United States
Resource Type: Journal article
Publication Details: The Laryngoscope, Vol.122(9), pp.1898-1905
DOI: 10.1002/lary.23392
PMID: 22711071
PMCID: PMC3449319
NLM abbreviation: Laryngoscope
ISSN: 0023-852X
eISSN: 1531-4995
Publisher: Wiley-Blackwell; Hoboken, NJ
Language: English
Date published: 2012
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Neurology; Radiology; Molecular Physiology and Biophysics; Pulmonary, Critical Care, and Occupational Medicine; Microbiology and Immunology; Pulmonary Medicine; Stead Family Department of Pediatrics; Pathology; Iowa Neuroscience Institute; Neurosurgery; Internal Medicine
Record Identifier: 9984020604702771

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