2019 Webinar: New Insights Into the Pathogenesis and Management of Cystic Fibrosis-Related Liver Disease-New Insights Into the Pathogenesis and Management of Cystic Fibrosis-Related Liver Disease

New Insights Into the Pathogenesis and Management of Cystic Fibrosis-Related Liver Disease

Video Transcription

Video Summary

This video highlights the pathogenesis, management, and treatment options for advanced liver disease in patients with cystic fibrosis. It discusses the potential mechanisms of injury, including CFTR dysfunction and obliterated portal venopathy, that lead to fibrosis and cirrhosis. The role of the microbiome in CF liver disease is also explored. The video emphasizes the need for better biomarkers for CF liver disease and presents potential options such as APRI scores, platelet count, and elastography. Treatment options for advanced CF liver disease are discussed, including splenic embolization, splenectomy, shunt procedures, and liver transplantation. The use of CFTR modulator therapies is also reviewed, with an emphasis on their potential to improve liver function. The video concludes by acknowledging the need for further research on the effects of these therapies on CF liver disease. Overall, the video provides a comprehensive overview of the diagnosis and management of advanced liver disease in cystic fibrosis patients, presenting insights into potential causes, diagnostic strategies, treatment options, and the exciting new possibilities offered by CFTR modulator therapies.

Asset Caption

Presenter: A. Jay Freeman, MD; Daniel H. Leung, MD, FAASLD and Michael R. Narkewicz, MD, FAASLD
Moderator: Daniel H. Leung, MD, FAASLD and Michael R. Narkewicz, MD, FAASLD

Keywords

advanced liver disease

cystic fibrosis

CFTR dysfunction

fibrosis

cirrhosis

microbiome

biomarkers

treatment options

liver transplantation

CFTR modulator therapies

diagnosis