Update on the Management of Chronic Rhinosinusitis in Patients with Cystic Fibrosis: A Literature Review
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Keywords
cystic fibrosis, rhinosinusitis, CFTR modulator, Endoscopic sinus surgery
Abstract
Cystic fibrosis is an autosomal recessive genetic disease caused by mutations in the CFTR channel. These mutations determines an alteration in the exchange of chloride and bicarbonate, leading to thickening of mucus and subsequent mucociliary dysfunction. This results in multisystemic symptoms, with pulmonary manifestations being of primary concern. While sinus symptoms may not manifest universally among individuals with cystic fibrosis, radiological prevalence of 100% is described. Beyond contributing to systemic morbidity, these symptoms serve as a reservoir for bacteria and a source of pulmonary exacerbations. Clinically, it has been observed that these patients are refractory to conventional therapies used for chronic rhinosinusitis. This literature review describes different treatments proposed for this population: nasal saline lavage, intranasal corticosteroids, topical antibiotics, CFTR modulators, endoscopic sinus surgery.
