Cystic fibrosis medical therapy
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]
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Overview
Medical Therapy
- Inhaled osmotic agent/ Hypertonic saline: Increase the airway fluid layer [1]
- Dornase alfa [recombinant human deoxyribo-nuclease (DNase)]: Cleave the extracellular DNA and aid airway clearance (DNA significantly increases the viscosity of the sputum) [2]
| Summary table of medical treatment in patients with Cystic fibrosis | ||
|---|---|---|
| Category | Approaches | Features |
| Mucolytic agents | Dornase alfa | Cleave the extracellular DNA and aid airway clearance |
| N-acetyl-L-cysteine | Also increase levels of the intracellular antioxidant glutathione (GSH) that protect against the neutrophil-driven tissue damage | |
| Airway surface rehydration | Hypertonic saline | As it may cause bronchoconstriction, it is commonly used with an bronchodilator |
| Osmotic agents | Mannitol is a nonabsorbable sugar alcohol which provides an osmotic gradient on the airway surface | |
| Correction of ion transport | ||
| Anti-infective agents | Prophylaxis | |
| Eradication | ||
| Suppression | ||
| Acute exacerbations | ||
| Inflammation | Anti-inflammatory agents | |
| Inhaled corticosteroids | ||
| LTB4 receptor antagonists | ||
| Azithromycin | ||
| CFTR protein defect | Potentiators | |
| Correctors and combination
therapy |
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References
- ↑ Ratjen FA (2009). "Cystic fibrosis: pathogenesis and future treatment strategies". Respir Care. 54 (5): 595–605. PMID 19393104.
- ↑ Edmondson C, Davies JC (2016). "Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications". Ther Adv Chronic Dis. 7 (3): 170–83. doi:10.1177/2040622316641352. PMC 4907071. PMID 27347364.