Gamma‐glutamyl transferase and disease course in pediatric‐onset primary sclerosing cholangitis: A single‐center cohort study

• Published in: Health science reports Vol. 6; no. 2; pp. e1086 - n/a
• Main Authors: Berhane, Besrat, Rheenen, Patrick F., Verkade, Henkjan J.
• Format: Journal Article
• Language: English
• Published: United States John Wiley & Sons, Inc 01.02.2023; John Wiley and Sons Inc; Wiley
• Subjects: Adults; Ascites; autoimmune hepatitis; Bile ducts; Cholangitis; Clinical outcomes; Cohort analysis; Ethics; hepatic complications; Histology; Hypertension; Inflammatory bowel disease; Laboratories; Liver diseases; liver transplantation; Liver transplants; Original Research; Patients; Pediatrics; Review boards; surrogate marker; clinical outcomes; hepatic complications; autoimmune hepatitis; surrogate marker; liver transplantation
• ISSN: 2398-8835; 2398-8835
• DOI: 10.1002/hsr2.1086

Background and Aims Patients with pediatric‐onset primary sclerosing cholangitis (PSC) are at risk of developing hepatic complications with liver transplantation as only curative treatment. Complications usually occur over many years, underlining the need for reliable surrogate markers to predict the clinical course. Recently, gamma‐glutamyl transferase (GGT) has been suggested to allow prediction of the clinical course. In a single‐center cohort study, we tested the potency of GGT in this respect. Methods We used longitudinal data of patients from our academic center, diagnosed with pediatric‐onset PSC between 2000 and 2020. Patients with a GGT decrease from baseline >25% (n = 36) were compared with those who did not have this decrease (n = 7). We performed Kaplan–Meier analysis and log‐rank testing to assess the occurrence of portal hypertensive or biliary complications, hepatobiliary malignancies, liver transplantation, or death. Results The median age diagnosis was 15.2 years and 12.1 years in the group with ≤25% decrease of GGT and the group with >25% decrease, respectively (p = 0.078). The probability of developing ≥1 complications in the first 5 years after diagnosis was 50% in the group with ≤25% decrease of GGT and 20% in the group with >25% decrease of GGT (p = 0.031). The use of medication was not associated with the development of complications. Conclusion In a retrospective cohort study, we report that a GGT decrease of >25% within 1 year of diagnosis of pediatric‐onset PSC is associated with a lower occurrence of complications within 5 years. Our results provide further support for the recently hypothesized predictive value of first‐year GGT change in predicting the disease course in pediatric‐onset PSC.