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Pathophysiology of Cystic Fibrosis Liver Disease

Vania L Kasper1, David N Assis2

  • 1The Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Hasbro Children's Hospital, Warren Alpert Medical School of Brown University, Providence, Rhode Island, USA.

Pediatric Pulmonology
|August 6, 2024
PubMed

Insights

Cystic Fibrosis (CF) can cause serious liver and bile duct problems, leading to death in advanced cases. Understanding CF

Area of Science:

  • Hepatology and Gastroenterology
  • Genetics and Molecular Biology
  • Pulmonology and Respiratory Medicine

Background:

  • Hepatobiliary complications are a major health burden in Cystic Fibrosis (CF).
  • Advanced CF liver disease is a leading cause of mortality among CF patients.
  • The diverse clinical presentations, from steatosis to biliary strictures, have poorly understood origins.

Purpose of the Study:

  • To review the current understanding of the pathophysiology of hepatobiliary manifestations in CF.
  • To discuss emerging disease models for CF-related liver disease.
  • To explore promising therapeutic strategies for this aspect of CF care.

Main Methods:

  • Literature review of existing research on CF hepatobiliary complications.
  • Analysis of current knowledge on CFTR mutations and their impact on liver health.
  • Examination of novel preclinical and clinical studies on CF liver disease.

Main Results:

  • Hepatobiliary complications in CF are complex and multifactorial.
  • Underlying CFTR mutations interact with environmental and modifying factors.
  • Current understanding of pathophysiology is incomplete, necessitating further research.
  • Emerging models and therapies show potential for improved patient outcomes.

Conclusions:

  • Hepatobiliary manifestations are a critical concern in CF care.
  • Further research into pathophysiology and therapeutic targets is essential.
  • Improved understanding and treatment of CF liver disease can reduce mortality.

Keywords:
Cystic Fibrosisbiliaryliverorganoidpathophysiology

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