More from Aspen Webinar 2021. This blog entry has abbreviated/summarized several presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well. Great lecture from Jim Squires.

Key points:

• Cystic Fibrosis Liver Disease (CFLD) is variably defined
• Risk factors include male patients, DeltaF508 mutations, meconium ileus and SERPINA1 Z allele
• Two main phenotypes: Classic “Focal Biliary Cirrhosis” and Obliterative Portal Venopathy (increasingly recognized)
• Intestinal microbiome and gut permeability/endotoxins may influence liver disease
• Treatments: ursodeoxycholic acid may be helpful but overall evidence is low quality. Cochrane review does NOT recommend its routine use
• Treatments: liver transplant (thorough review: Freeman et al. Liver Transpl 2019; 25: 640-657)
• Treatments: CF Modulators: potentiators (Ivacaftor), correctors (Lumacaftor, Elexacaftor, Tezacaftor)
• Treatment: Trikafta has been a game changer for CF lung disease. Its effects on the liver are not clear yet

Some of the slides:

Related blog posts:

• CHOA Nutrition Support -CF in the age of “Miracle Drug”
• Data on Immobilized Lipase Cartridge for Patients with CF
• Cystic Fibrosis Expert Update 2017
• Nutrition Pearls: Fiber and Cystic Fibrosis
• Big Advance for Cystic Fibrosis -Who Will Benefit?
• Nutrition Guidelines for Cystic Fibrosis
• Complex Family of CFTR-Associated Disorders | gutsandgrowth

SAVE THE DATE for next year’s conference: July 11-15, 2022 in Snowmass Village, CO