Journal article
Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor
International forum of allergy & rhinology, Vol.5(2), pp.178-181
02/2015
DOI: 10.1002/alr.21440
PMCID: PMC4310814
PMID: 25363320
Abstract
Background
Chronic sinusitis is universal in cystic fibrosis (CF) and our current treatments are ineffective in reversing sinus disease. The objective of this work was to determine if increasing CF transmembrane conductance regulator (CFTR) activity by ivacaftor could treat CF sinus disease and assess its effect on primary sinus epithelial cultures.
Methods
Case report of 1 patient with long‐standing chronic sinus disease and a new diagnosis of CF with a mild mutation (P205S) and a severe mutation (G551D). We discuss clinical changes in symptoms, radiographic findings, nasal potential difference testing, and nasal pH values before and after treatment with ivacaftor. We then developed primary sinonasal epithelial cell cultures from a biopsy of the patient to determine changes in airway surface liquid (ASL) pH and ASL viscosity after ivacaftor treatment.
Results
Ivacaftor treatment reversed CT findings of CF sinus disease, increased nasal voltage and pH, and resolved sinus symptoms after 10 months of therapy. Ivacaftor significantly increased ASL pH and decreased ASL viscosity in primary airway cultures.
Conclusion
This report documents the reversal of CF sinus disease. Based on our in vivo and in vitro results, we speculate that ivacaftor may reverse CF sinusitis by increasing ASL pH and decreasing ASL viscosity. These studies suggest that CFTR modulation may be effective in treating CF and perhaps non‐CF sinusitis.
Details
- Title: Subtitle
- Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor
- Creators
- Eugene H Chang - University of IowaXiao Xiao Tang - University of IowaViral S Shah - University of IowaJanice L Launspach - University of IowaSarah E Ernst - University of IowaBrieanna Hilkin - University of IowaPhilip H Karp - University of IowaMahmoud H Abou Alaiwa - University of IowaScott M Graham - University of IowaDouglas B Hornick - University of IowaMichael J Welsh - University of IowaDavid A Stoltz - University of IowaJoseph Zabner - University of Iowa
- Resource Type
- Journal article
- Publication Details
- International forum of allergy & rhinology, Vol.5(2), pp.178-181
- DOI
- 10.1002/alr.21440
- PMID
- 25363320
- PMCID
- PMC4310814
- NLM abbreviation
- Int Forum Allergy Rhinol
- ISSN
- 2042-6976
- eISSN
- 2042-6984
- Number of pages
- 4
- Grant note
- Cystic Fibrosis Foundation (CHANG13IO (EHC) NIH‐NIDCR (DE021413‐01A1 (EHC)
- Language
- English
- Date published
- 02/2015
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Neurology; Molecular Physiology and Biophysics; Pulmonary, Critical Care, and Occupational Medicine; Neurosurgery; Otolaryngology; Internal Medicine
- Record Identifier
- 9984013203202771
Metrics
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