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Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
Nasal and sinus disease in cystic fibrosis
John M Robertson1, Ellen M Friedman, Bruce K Rubin
1Department of Pediatrics, Texas Children's Hospital, Houston, Texas 77030, USA.
Insights
Cystic fibrosis (CF) patients frequently have paranasal sinus disease, often without symptoms. Early diagnosis and management of CF sinus disease may improve overall health and lung transplant outcomes.
Area of Science:
- Otolaryngology
- Pulmonology
- Genetics
Background:
- Paranasal and sinus disease is nearly universal in cystic fibrosis (CF) patients.
- Symptoms are often absent, delaying diagnosis and treatment.
- Disease characteristics like polyposis and microbial flora evolve with age.
Purpose of the Study:
- To review the current understanding of paranasal sinus disease in CF.
- To highlight diagnostic advancements and the spectrum of sinonasal abnormalities.
- To discuss treatment options and their impact on overall health.
Main Methods:
- Review of current literature on CF-related sinonasal disease.
- Analysis of findings from endoscopic and CT imaging.
- Evaluation of existing treatment data, including surgical outcomes.
Main Results:
- Endoscopy and CT scans reveal a high prevalence of polyposis and specific anatomical abnormalities.
- Age-related changes in sinonasal disease are observed.
- Limited evidence exists for medical and surgical treatments, with no clear selection criteria for surgery.
Conclusions:
- CF sinus disease is common and complex, with significant anatomical changes.
- Sinus surgery may offer benefits, but further research is needed.
- Effective management of sinonasal disease may positively impact lung function and survival post-transplant.
Abstract:
Paranasal and sinus disease is present in almost every patient with cystic fibrosis. However, symptoms are rarely reported. Some aspects including polyposis and microbial flora change with patient age. Endoscopy and computerised tomography have broadened our understanding of how this disease affects the sinuses, including an increased recognition of polyposis than previously thought and identification of several disease specific entities such as abnormalities of the lateral nasal wall and uncinate process. Few randomised, controlled trials evaluating medical or surgical treatments of CF sinus disease exist. Sinus surgery may provide some benefit, though there are no established selection criteria for appropriate candidates. A link between sinus disease and lower respiratory tract function may contribute to general health and survival following lung transplantation. Complications of sinonasal disease in CF are rare and include mucoceles and periorbital abscesses.
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