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Liver transplantation for primary sclerosing cholangitis in children versus young adults
Ioannis A Ziogas1, Lauren Maloney2, Dor Yoeli3
1Division of Transplant Surgery, Department of Surgery, University of Colorado Anschutz Medical Campus, Children's Hospital Colorado, Aurora, CO.
Insights
Children with primary sclerosing cholangitis (PSC) have better patient survival after liver transplantation than young adults. However, disparities in liver transplant outcomes persist based on race and ethnicity.
Area of Science:
- Hepatology
- Transplant Surgery
- Immunology
Background:
- Primary sclerosing cholangitis (PSC) is an immune-mediated cholestatic liver disease.
- PSC is characterized by bile duct inflammation and is associated with inflammatory bowel disease (IBD).
Purpose of the Study:
- To compare outcomes of liver transplantation for PSC in children versus young adults.
- To identify factors influencing patient and graft survival post-transplantation.
Main Methods:
- Retrospective analysis of United Network for Organ Sharing data (2002-2024).
- Comparison of pediatric (<18 years) and young adult (18-25 years) liver transplant recipients with PSC.
- Multivariable Cox regression analysis to assess patient and graft survival.
Main Results:
- Children had lower MELD/PELD scores and less IBD than young adults.
- Pediatric recipients showed decreased risk of patient mortality (HR 0.49) and White, non-Hispanic patients had improved survival.
- Female sex and White, non-Hispanic race/ethnicity were associated with decreased graft loss risk.
Conclusions:
- Pediatric liver transplant recipients with PSC have superior patient survival compared to young adults.
- Graft survival did not differ significantly between age groups.
- White, non-Hispanic race/ethnicity is linked to improved survival, highlighting disparities in liver transplantation.
Introduction:
Primary sclerosing cholangitis is an immune-mediated cholestatic liver disease characterized by inflammation of the intra- and/or extrahepatic bile ducts and is associated with inflammatory bowel disease.
Methods:
We retrospectively compared children (<18 years) and young adults (18-25 years) undergoing first liver transplantation for primary sclerosing cholangitis using United Network for Organ Sharing data (February 2002-December 2024).
Results:
A total of 531 patients with primary sclerosing cholangitis underwent liver transplantation (160 children; 371 young adults). Children had lower laboratory Model for End-stage Liver Disease / Pediatric End-stage Liver Disease score than young adults (median 13.0 vs 20.0, P < .001). A smaller proportion of children had inflammatory bowel disease (58.8% vs 67.9%, P = .04), ascites (35.9% vs 47.8%, P = .01), and underwent living donor liver transplantation (18.1% vs 29.9%, P < .001) than young adults. Multivariable Cox regression showed decreased risk of patient mortality in children versus young adults (hazard ratio 0.49, 95% confidence interval 0.27-0.89, P = .02) and White, non-Hispanic patients compared with other race/ethnicity (0.44, 0.27-0.73, P = .001), when adjusting for inflammatory bowel disease, Model for End-stage Liver Disease / Pediatric End-stage Liver Disease score, intensive care unit status, graft type, and era. Multivariable Cox regression showed decreased risk of graft loss in female versus male sex (hazard ratio 0.68, 95% confidence interval 0.49-0.96, P = .03) and White, non-Hispanic patients compared with other race/ethnicity (0.71, 0.50-0.99, P = .04), when adjusting for inflammatory bowel disease, Model for End-stage Liver Disease / Pediatric End-stage Liver Disease score, intensive care unit status, graft type, and era.
Conclusion:
Pediatric liver transplantation recipients with primary sclerosing cholangitis exhibit superior patient survival versus young adult liver transplantation recipients, whereas graft survival does not differ significantly. White, non-Hispanic race/ethnicity was independently associated with improved survival, underscoring persistent disparities in liver transplantation outcomes.
