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Elevated Serum Bile Acids Predict Poor Liver Outcomes in Children With Alagille Syndrome: Results From the GALA Study

Carla Fiorella Murillo Perez1, Shannon M Vandriel1, Emmanuel M Gonzales2

  • 1Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children and the University of Toronto, Toronto, Canada.

Liver International : Official Journal of the International Association for the Study of the Liver
|November 18, 2025
PubMed

Insights

Lower serum bile acid (SBA) levels in children with Alagille syndrome (ALGS) predict better liver disease outcomes, including native liver survival (NLS) and event-free survival (EFS). These findings suggest that reducing SBA may improve clinical outcomes in ALGS patients.

Area of Science:

  • Hepatology
  • Pediatric Gastroenterology
  • Rare Diseases

Background:

  • Alagille syndrome (ALGS) is a genetic disorder causing liver disease and other health issues.
  • Serum bile acid (SBA) levels are a key indicator in cholestatic liver diseases.
  • Emerging therapies target the ileal bile acid transporter (IBAT) to lower SBA.

Purpose of the Study:

  • To investigate if SBA levels predict liver disease outcomes in children with ALGS.
  • To assess the prognostic value of SBA in relation to native liver survival (NLS) and event-free survival (EFS).

Main Methods:

  • Utilized data from the Global ALagille Alliance (GALA) cohort.
  • Employed Cox regression analysis with a time-dependent covariate for SBA.
  • Assessed a prognostic SBA threshold of 102 μmol/L, adjusting for total bilirubin (TB).

Main Results:

  • A moderate positive correlation was observed between SBA and TB (r=0.47, p<0.001).
  • SBA levels below 102 μmol/L significantly predicted improved NLS and EFS (p<0.001).
  • Lower SBA remained a significant predictor of improved EFS even after adjusting for TB clearance at 1 year.

Conclusions:

  • Reduced SBA in ALGS patients is associated with better NLS and EFS.
  • SBA also correlates with NLS in ALGS patients who achieve anicteric cholestasis.
  • Lowering SBA may represent a therapeutic strategy to improve clinical outcomes in ALGS.
Abstract

Keywords:
IBATcholestasispaediatrics

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