Related Experiment Video
Updated: Oct 20, 2026
Complete Laparoscopic Radical Resection of Perihilar Cholangiocarcinoma Type IIIb
Published on: January 17, 2025
Primary sclerosing cholangitis (PSC): clinical, laboratory and survival analysis in children and adults
A Floreani1, L Zancan, A Melis
1Department of Gastroenterological and Surgical Sciences, University of Padova, Italy.
Insights
Primary sclerosing cholangitis (PSC) is more active in children but has a better prognosis than in adults. Adults with PSC experienced more severe outcomes, including liver failure and cancer, while children showed no deaths during the study period.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Autoimmune Diseases
Background:
- Primary sclerosing cholangitis (PSC) is a rare pediatric liver disease with limited natural history and survival data.
- Diagnosis in children is uncommon, necessitating further research into disease characteristics and outcomes.
Purpose of the Study:
- To compare clinical, laboratory, and survival rates of pediatric PSC (Group A) versus adult PSC (Group B).
- To elucidate differences in disease presentation and long-term outcomes between pediatric and adult PSC cohorts.
Main Methods:
- Retrospective analysis of 9 pediatric (mean age 10) and 28 adult (mean age 32) PSC patients.
- Follow-up averaged 5.2 years for children and 6.9 years for adults.
- Kaplan-Meier survival analysis was employed, with ERCP and colonoscopy performed in all cases.
Main Results:
- Pediatric PSC patients presented with higher IgG and AST levels and a higher incidence of interface hepatitis.
- Adults experienced significant adverse events including liver failure (n=6), cholangiocarcinoma (n=3), and colonic cancer (n=1).
- Four adults required liver transplantation (OLTx), with one mortality post-retransplantation; no deaths occurred in the pediatric group.
Conclusions:
- Pediatric PSC exhibits more severe disease activity at presentation compared to adults.
- Despite initial severity, children with PSC demonstrate a better long-term prognosis than adults.
- Adult PSC patients faced a poorer outcome, with poor outcomes exclusively observed in this group.
Background:
Primary sclerosing cholangitis (PSC) is an uncommon disorder, rarely diagnosed in children, moreover, data on its natural history and survival are still lacking.
Aim:
The study was undertaken to compare clinical, laboratory and survival rates in two series of PSC: one in a pediatric group (group A) and the other in an adult population (group B).
Methods:
Group A included 9 patients (5 males, 4 females, mean age 10 yrs, range 7-15); group B included 28 patients (19 males, 9 females, mean age 32 years, range 19-60). The mean follow-up was 5.2 years in group A and 6.9 years in group B (range 1-14 years). ERCP and colonoscopy were performed in each case. Survival was analyzed using the Kaplan-Meier method.
Results:
At presentation children showed significantly higher levels of IgG and AST compared to adults (p<0.05); moreover, interface hepatitis occurred in 50% of children and in 14.2% in adults (p=ns). During follow-up the following major events occurred: oesophageal bleeding (n=2) in group A; progressive liver failure (n=6), cholangiocarcinoma (n=3), colonic cancer (n=1) in group B. Liver transplantation (OLTx) was performed in 4 adults (one died after a retransplantation). No deaths were observed in children. The Kaplan-Meier curve in adults shows a 65% rate of survival at 10 years.
Conclusions:
The present findings on PSC suggest a more severe activity of the disease in children than in adults at presentation; nonetheless, the prognosis seems to be better in children than in adults. The Mayo score prognostic index does not predict the development of liver/colonic cancer. A poor outcome (defined as death or being listed for OLTx) only occurred in adults.