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.