Faculty Collaboration Database

Lack of Correlation of Liver Tests With Fibrosis Stage at Diagnosis in Pediatric Primary Sclerosing Cholangitis. J Pediatr Gastroenterol Nutr 2018 Feb;66(2):227-233

Date

01/23/2018

Pubmed ID

29356767

DOI

10.1097/MPG.0000000000001789

Scopus ID

2-s2.0-85041590902 (requires institutional sign-in at Scopus site)   5 Citations

Abstract

OBJECTIVES: The aims of this study were to characterize pediatric primary sclerosing cholangitis (PSC) at a regional referral-based institution, including scoring of biliary stricturing and liver fibrosis and correlation analyses of scores with serum liver tests, to identify biomarkers of disease severity.

METHODS: A retrospective review of 39 PSC subjects was performed, with collection of demographic and outcomes data. Magnetic resonance cholangiopancreaticogram (MRCP) and liver biopsies were re-reviewed and scores of stricturing and fibrosis were correlated with serum liver tests.

RESULTS: Average age at PSC diagnosis was 11.2 years, 74% had inflammatory bowel disease and 51% had autoimmune hepatitis. Despite 83% with symptoms at presentation, only ∼1/3 were symptomatic at a mean follow-up of 4.1 years. Using a validated MRCP biliary scoring system, the mean intrahepatic score was 1.1 (out of 4) and extrahepatic score was 1.0 (out of 3). The mean Ishak liver fibrosis stage was 3.5 (out of 6) and 33% had cirrhosis. 92% were alive with their native liver and 5% had a liver transplant. Serum biomarker analyses revealed no correlation between Ishak liver fibrosis stage or MRCP score and laboratory values.

CONCLUSIONS: Pediatric PSC patients cared for at a regional referral center had relatively mild disease compared with previously published reports, with low MRCP stricture scores despite significant liver fibrosis. Liver tests at presentation did not correlate with MRCP stricture score or liver fibrosis stage, suggesting the need for future studies to identify potential biomarkers of disease severity.

Author List

Cotter JM, Browne LP, Capocelli KE, McCoy A, Mack CL

Author

Cara Lynn Mack MD Chief, Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Biliary Tract
Biomarkers
Child
Child, Preschool
Cholangiopancreatography, Magnetic Resonance
Cholangitis, Sclerosing
Female
Humans
Infant
Liver
Liver Cirrhosis
Liver Function Tests
Male
Retrospective Studies