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.
journal_name
Int Forum Allergy Rhinoljournal_title
International forum of allergy & rhinologyauthors
Chang EH,Tang XX,Shah VS,Launspach JL,Ernst SE,Hilkin B,Karp PH,Abou Alaiwa MH,Graham SM,Hornick DB,Welsh MJ,Stoltz DA,Zabner Jdoi
10.1002/alr.21440subject
Has Abstractpub_date
2015-02-01 00:00:00pages
178-81issue
2eissn
2042-6976issn
2042-6984journal_volume
5pub_type
杂志文章abstract:BACKGROUND:It has been found that the most common adverse reaction which occurs more frequently on cetirizine than on placebo is somnolence. However, the somnolence rate varied widely among different studies. The objective of this study was to assess the somnolence effect of cetirizine 10 mg daily compared to placebo i...
journal_title:International forum of allergy & rhinology
pub_type: 杂志文章,meta分析
doi:10.1002/alr.21746
更新日期:2016-08-01 00:00:00
abstract:BACKGROUND:Despite considerable research, the pathogenesis of chronic rhinosinusitis (CRS) remains poorly understood. Potential microbial roles in the etiology or progression of CRS have long been hypothesized, yet few specific associations have been identified. In this study we investigate associations between pattern...
journal_title:International forum of allergy & rhinology
pub_type: 杂志文章
doi:10.1002/alr.21871
更新日期:2017-03-01 00:00:00
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journal_title:International forum of allergy & rhinology
pub_type: 临床试验,杂志文章,多中心研究
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更新日期:2013-10-01 00:00:00
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journal_title:International forum of allergy & rhinology
pub_type: 杂志文章
doi:10.1002/alr.21476
更新日期:2015-04-01 00:00:00
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journal_title:International forum of allergy & rhinology
pub_type: 杂志文章
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更新日期:2012-03-01 00:00:00
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pub_type: 杂志文章
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更新日期:2015-04-01 00:00:00
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journal_title:International forum of allergy & rhinology
pub_type: 杂志文章
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更新日期:2015-10-01 00:00:00
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journal_title:International forum of allergy & rhinology
pub_type: 杂志文章
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更新日期:2017-12-01 00:00:00
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pub_type: 杂志文章
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更新日期:2018-07-01 00:00:00
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journal_title:International forum of allergy & rhinology
pub_type: 杂志文章
doi:10.1002/alr.21247
更新日期:2014-02-01 00:00:00
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pub_type: 共识发展会议,杂志文章
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更新日期:2018-02-01 00:00:00
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pub_type: 杂志文章
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更新日期:2016-05-01 00:00:00
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journal_title:International forum of allergy & rhinology
pub_type: 杂志文章
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pub_type: 杂志文章,随机对照试验
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更新日期:2018-01-01 00:00:00
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pub_type: 杂志文章
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更新日期:2018-06-01 00:00:00
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journal_title:International forum of allergy & rhinology
pub_type: 杂志文章,评审
doi:10.1002/alr.21386
更新日期:2014-09-01 00:00:00
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pub_type: 杂志文章
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更新日期:2018-11-01 00:00:00
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pub_type: 杂志文章
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更新日期:2016-02-01 00:00:00
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pub_type: 杂志文章,随机对照试验
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更新日期:2019-11-01 00:00:00
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pub_type: 杂志文章
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更新日期:2017-08-01 00:00:00
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journal_title:International forum of allergy & rhinology
pub_type: 杂志文章
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更新日期:2016-02-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2013-02-01 00:00:00
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pub_type: 杂志文章
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更新日期:2015-12-01 00:00:00
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pub_type: 杂志文章
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更新日期:2015-01-01 00:00:00
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journal_title:International forum of allergy & rhinology
pub_type: 杂志文章
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更新日期:2016-02-01 00:00:00
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pub_type: 杂志文章
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更新日期:2017-07-01 00:00:00