Chloride Secretagogues for Acquired CFTR Dysfunction in Chronic Rhinosinusitis (NPD Protocol)
The purpose of this study is to determine if acquired (partial) Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) deficiency contributes substantially to the pathogenic mechanisms underlying Chronic Rhinosinusitis (CRS), creating a localized environment that impairs mucociliary clearance (MCC).
⁃ a. Patients with CRS will be diagnosed according to Sinus and Allergy Health Partnership symptom-based and objective criteria as follows: i. Duration of disease is qualified by continuous symptoms (≥ 2 major factors or at least 1 major factor \& 2 minor symptoms; Table 2) for ≥ 12 consecutive weeks or ≥ 12 weeks of physical findings. ii. One of these signs of inflammation must be present and identified in association with ongoing symptoms.
• Discolored nasal drainage arising from the nasal passages, nasal polyps, or polypoid swelling as identified on physical examination with nasal endoscopy.
• Edema or erythema of the middle meatus or ethmoid bulla
• Generalized or localized erythema or edema. If it does not involve the middle meatus or ethmoid bulla, CT scan is performed to confirm a diagnosis.
• The CT scan must demonstrate isolated or diffuse mucosal thickening, bone changes, air-fluid levels. b. Age ≥ 19 years and Weight ≥ 50 kg c. Ability to perform NPD testing d. Negative pregnancy test (for females of childbearing potential) e. Written informed consent