Cystic Fibrosis

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What is the pathology pathway and relevant therapeutic strategies?

1. Defective CF gene -- gene therapy 2. Dysfunctional CFTR -- CFTR correctors/potentiators 3. decreased Cl- secretion -- CFTR bypass therapy 4. mucus accumulation/bronchial obstruction -- airway clearance, bronchodilators, mucolytics 5. infection -- antibiotics 6. inflammation -- antiinflammatories 7. bronchiectasis -- lung transplant





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