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Published on: June 5, 2014
Recurrence of Primary Sclerosing Cholangitis After Pediatric Liver Transplantation: A Single-Center, Retrospective
Athaya Vorasittha1, Seisuke Sakamoto1, Yusuke Yanagi1
1Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
Insights
Recurrent primary sclerosing cholangitis (rPSC) affects 30.7% of pediatric liver transplant recipients, often occurring in younger patients with inflammatory bowel disease. Immunologic interventions may help prevent rPSC, warranting further research.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Transplant Surgery
Background:
- Primary sclerosing cholangitis (PSC) is a challenging condition in pediatric liver transplantation (LT).
- Recurrent PSC (rPSC) after LT significantly impacts long-term outcomes.
- Identifying risk factors for rPSC is crucial for improving pediatric LT success rates.
Purpose of the Study:
- To characterize pediatric liver transplant recipients with PSC.
- To identify potential risk factors associated with the recurrence of PSC (rPSC) post-transplantation.
- To evaluate outcomes in pediatric LT recipients with and without rPSC.
Main Methods:
- Retrospective analysis of 13 pediatric patients undergoing LT for PSC at a single center.
- Comparison of patient characteristics, risk factors, and outcomes between groups with and without rPSC.
- Assessment of demographic data, co-existing conditions (IBD, AIH), graft type, and post-transplant complications.
Main Results:
- The recurrence rate of PSC (rPSC) was 30.7% (4/13 patients) within a median follow-up of 53 months.
- Patients who developed rPSC were younger at PSC diagnosis and universally experienced acute cellular rejection (ACR).
- rPSC was associated with inflammatory bowel disease (IBD) and autoimmune hepatitis (AIH) overlap, though not statistically significant.
Conclusions:
- Pediatric liver transplantation for PSC has a high rate of recurrence (rPSC).
- Immune-activating conditions like IBD and AIH may be linked to rPSC development.
- Further prospective studies are needed to explore immunologic interventions for rPSC prevention.
Aim:
Liver transplantation (LT) is a well-accepted treatment for primary sclerosing cholangitis (PSC) with generally good outcomes, although recurrent PSC (rPSC) poses significant challenges. This study aimed to describe patient characteristics and identify potential risk factors of rPSC in pediatric LT recipients.
Methods:
This retrospective study analyzed 13 pediatric patients who underwent LT for PSC at a single center. Patient characteristics, risk factors, and outcomes were compared between those with and without rPSC.
Results:
The median age at PSC diagnosis was 5.2 years and at LT, 15.4 years. Inflammatory bowel disease (IBD) was present in 12 patients (92.3%), and four (30.7%) had overlapping autoimmune hepatitis (AIH) before LT. Two patients received grafts from living-related donors, and 11 from deceased donors. During a median follow-up of 53 months, 4 of the 13 patients (30.7%) developed rPSC at a median of 48.9 months post-LT. Patients with rPSC tend to be younger at PSC diagnosis. All rPSC cases were associated with IBD, and half had AIH overlap, though the frequency difference was not significant. Acute cellular rejection (ACR) was universal in rPSC patients (100%) compared to nonrecurrent cases (33.3%, p = 0.07). One case of rPSC developed pulmonary hypertension following rPSC and succumbed to PH crisis, resulting in a 5-year patient survival rate of 82%.
Conclusions:
The recurrence rate was high in pediatric patients with PSC. The observed association with immune-activating conditions raises the possibility of utilizing immunologic interventions to prevent rPSC, although further prospective studies are warranted to clarify the underlying mechanisms.
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