MTE #36 Special Considerations in the Management of Cystic Fibrosis Liver Disease

Description

Cystic fibrosis hepatobiliary involvement (CFHBI) occurs in up to 90% of people with cystic fibrosis. Advanced cystic fibrosis liver disease is present in approximately 10% of people with cystic fibrosis. CFHBI is the third leading cause of morbidity and mortality in people with cystic fibrosis; early diagnosis and surveillance for disease progression are essential to optimize patient outcomes. Experts conducting this session review the current guidelines for monitoring CFHBI; the algorithm of care related to cystic fibrosis transmembrane conductance regulator protein modulator therapy hepatotoxicity; and the diagnositic and therapeutic options for managing advanced cystic fibrosis liver disease.