Mucus and inflammation in the HEMT Era: Persistent barriers to complete airway normalization in cystic fibrosis - PubMed
4 hours ago
- #Cystic Fibrosis
- #Mucus
- #Inflammation
- Cystic fibrosis (CF) is an epithelial ion transport disorder causing airway dehydration and mucus concentration.
- CFTR dysfunction and microenvironmental cues like hypoxia and IL-1 signaling contribute to mucus hyperconcentration and inflammation.
- Highly effective CFTR modulators like elexacaftor/tezacaftor/ivacaftor improve hydration and mucociliary clearance but do not fully normalize airways.
- Residual mucus rheologic abnormalities, inflammation, and infections persist despite CFTR restoration.
- Advanced methods like FRAP and micro-wire rheology help study mucus viscosity and transport in patient-derived cultures.
- An integrated therapeutic approach combining CFTR correction with mucus-targeted and anti-inflammatory strategies is proposed.