Aspen Webinar 2021 Part 7 -Cystic Fibrosis Liver Disease

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:

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

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