Abstract
Cystic fibrosis (CF) is a common autosomal recessive genetic disorder where a deletion mutation and subsequent downstream alteration in transmembrane regulator proteins results in increased mucus viscosity. CF manifests clinically with chronic multisystem inflammation and recurrent infections. Nearly all children with CF have chronic sinusitis, and a large majority will have concurrent sinonasal polyposis. Chronic sinusitis and sinonasal polyposis in pediatric patients with CF can be managed conservatively initially, though most will fail medical management and require surgical intervention. Unfortunately, symptom resolution is marginal and polyp recurrence rates are high. Currently, no cure exists for CF and the mainstay of treatment is to provide symptomatic relief, and minimize disease morbidity.
Keywords: Pediatric, cystic fibrosis, rhinosinusitis, sinusitis.
Current Pediatric Reviews
Title:Rhinosinusitis in the Pediatric Patient with Cystic Fibrosis
Volume: 10 Issue: 3
Author(s): Christopher Fundakowski, Rosemary Ojo and Ramzi Younis
Affiliation:
Keywords: Pediatric, cystic fibrosis, rhinosinusitis, sinusitis.
Abstract: Cystic fibrosis (CF) is a common autosomal recessive genetic disorder where a deletion mutation and subsequent downstream alteration in transmembrane regulator proteins results in increased mucus viscosity. CF manifests clinically with chronic multisystem inflammation and recurrent infections. Nearly all children with CF have chronic sinusitis, and a large majority will have concurrent sinonasal polyposis. Chronic sinusitis and sinonasal polyposis in pediatric patients with CF can be managed conservatively initially, though most will fail medical management and require surgical intervention. Unfortunately, symptom resolution is marginal and polyp recurrence rates are high. Currently, no cure exists for CF and the mainstay of treatment is to provide symptomatic relief, and minimize disease morbidity.
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Cite this article as:
Fundakowski Christopher, Ojo Rosemary and Younis Ramzi, Rhinosinusitis in the Pediatric Patient with Cystic Fibrosis, Current Pediatric Reviews 2014; 10 (3) . https://dx.doi.org/10.2174/1573396309666131209205748
DOI https://dx.doi.org/10.2174/1573396309666131209205748 |
Print ISSN 1573-3963 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-6336 |
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