Monitoring for signs of portal hypertension is essential. Limited evidence supports ursodeoxycholic acid (UDCA) efficacy in halting CF liver disease progression. The effect of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on liver outcomes lacks definitive data, since patients with CF-related liver disease were excluded from trials due to potential hepatotoxicity. A proposed approach involves using UDCA and modulators in early stages, along with anti-inflammatory agents, with further therapeutic strategies awaiting randomized trials. Prevention of portal hypertensive bleeding includes endoscopic sclerotherapy or ligation of esophageal varices. Nonselective beta-blockers may also prevent bleeding and could be cautiously implemented. Other non-etiological treatments require investigation.
Keyphrases
- cystic fibrosis
- blood pressure
- pseudomonas aeruginosa
- small molecule
- lung function
- anti inflammatory
- atrial fibrillation
- electronic health record
- ultrasound guided
- transcription factor
- angiotensin converting enzyme
- big data
- machine learning
- climate change
- locally advanced
- skeletal muscle
- angiotensin ii
- air pollution
- artificial intelligence
- arterial hypertension
- rectal cancer