A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis-Associated Liver Disease

Liver Transpl. 2019 Apr;25(4):640-657. doi: 10.1002/lt.25421. Epub 2019 Mar 20.

Abstract

Approximately 5%-10% of patients with cystic fibrosis (CF) will develop advanced liver disease with portal hypertension, representing the third leading cause of death among patients with CF. Cystic fibrosis with advanced liver disease and portal hypertension (CFLD) represents the most significant risk to patient mortality, second only to pulmonary or lung transplant complications in patients with CF. Currently, there is no medical therapy to treat or reverse CFLD. Liver transplantation (LT) in patients with CFLD with portal hypertension confers a significant survival advantage over those who do not receive LT, although the timing in which to optimize this benefit is unclear. Despite the value and efficacy of LT in selected patients with CFLD, established clinical criteria outlining indications and timing for LT as well as disease-specific transplant considerations are notably absent. The goal of this comprehensive and multidisciplinary report is to present recommendations on the unique CF-specific pre- and post-LT management issues clinicians should consider and will face.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Biopsy
  • Child
  • Child, Preschool
  • Chloride Channel Agonists / administration & dosage
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / mortality
  • Cystic Fibrosis / therapy
  • Female
  • Follow-Up Studies
  • Humans
  • Hypertension, Portal / diagnosis
  • Hypertension, Portal / etiology
  • Hypertension, Portal / mortality
  • Hypertension, Portal / therapy*
  • Infant
  • Liver / diagnostic imaging
  • Liver / pathology
  • Liver Cirrhosis / diagnosis
  • Liver Cirrhosis / etiology
  • Liver Cirrhosis / mortality
  • Liver Cirrhosis / therapy*
  • Liver Transplantation / methods
  • Liver Transplantation / standards*
  • Liver Transplantation / statistics & numerical data
  • Lung Transplantation / methods
  • Lung Transplantation / standards*
  • Lung Transplantation / statistics & numerical data
  • Male
  • Middle Aged
  • Patient Selection
  • Postoperative Care / methods
  • Postoperative Care / standards
  • Practice Guidelines as Topic
  • Preoperative Care / methods
  • Preoperative Care / standards
  • Referral and Consultation / standards
  • Severity of Illness Index
  • Survival Rate
  • Time Factors
  • Time-to-Treatment
  • Treatment Outcome
  • Young Adult

Substances

  • Chloride Channel Agonists