The goal of this study is to execute a small clinical proof of concept trial: To examine the effects of losartan on mucociliary clearance (MCC) in patients not eligible for CFTR rescue therapies
Name: Losartan
Description: Losartan (100 mg for >12 weeks) will improve MCC+CC clearance in CF patients not on CFTR augmentation therapy in % of baseline
Measure: Improvement of mucociliary clearance ( MCC) and cough clearance (CC) Time: 12 weeks treatmentDescription: forced expiratory volume at one second (FEV1) in %predicted
Measure: Improvement on pulmonary function tests (in %predicted) Time: 12 weeks of treatmentDescription: forced expiratory volume at one second (FEV1) in liters
Measure: Improvement on pulmonary function tests (in L) Time: 12 weeks of treatmentDescription: Changes in serum inflammatory markers (hsCRP, WBC including absolute neutrophil count, %PMNs, serum amyloid A or SAA, calprotectin, GM-CSF, TGF-β active and total)
Measure: Decrease of inflammatory markers Time: 12 weeksDescription: Nasal cytokine changes (TGF-β1 active and total, TNF-α, IL-1β, IL-6, IL-8, IL-13, COX-2)
Measure: Nasal cytokine changes Time: 12 weeksAllocation: N/A
Single Group Assignment
There is one SNP
The CFTR potentiator ivacaftor (Kalydeco™), approved by the FDA mainly for class III mutations especially G551D, improves ion transport (large decrease in sweat chloride), clinical outcome (increased FEV1 and weight, decreased exacerbations), and quality of life. --- G551D ---