Good long-term outcomes of primary sclerosing cholangitis in childhood.

ABSTRACT:

Background & aims

Primary sclerosing cholangitis (PSC) is a rare progressive liver disease associated with inflammatory bowel disease (IBD). It is usually diagnosed in adults but can also present in children. Data on long-term outcomes of pediatric PSC are limited. Our aim was to study the natural history of pediatric PSC in Sweden.

Methods

This is a cohort study, including all children (<18 years), diagnosed with PSC between January 2000 and December 2015 at the Pediatric Liver Unit at Karolinska University Hospital, Stockholm. Patients were followed until liver transplantation, death or last date of follow-up (August 2021).

Results

We identified 124 children with a median age of 14 (1.9-17.8) years at PSC diagnosis. Sixty percent were boys, 93% had IBD. Median follo

SUBMITTER: Jerregard Skarby A 

PROVIDER: S-EPMC11321284 | biostudies-literature | 2024 Aug

REPOSITORIES: biostudies-literature