ArticleThe Journal of pediatrics2019
Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis: Predictors of Gamma Glutamyltransferase Normalization and Favorable Clinical Course.
Article in The Journal of pediatrics, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 2 of them syntheses that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
14 citing papers in PubMed, 2 syntheses or guidelines pooled it, 20 citations in OpenAlex.
- Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group.Journal of pediatric gastroenterology and nutrition · 2025Guideline
- [Guidelines on the diagnosis and management of primary sclerosing cholangitis (2021)].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2022Guideline
- The Childhood Liver Disease Research Network's prospective characterization of pediatric primary sclerosing cholangitis.Hepatology communications · 2026Observational
- Autoimmune liver diseases and overlap syndromes in children with inflammatory bowel diseases.European journal of pediatrics · 2026Review
- Potential Therapeutic Strategies for Steatosis, Oxidative Stress, Inflammation, and Fibrosis in Liver Disease.International journal of molecular sciences · 2026Review
- Primary sclerosing cholangitis in children: a single-center experience and evaluation of prognostic markers.Translational gastroenterology and hepatology · 2026Article
- Gamma-glutamyltransferase testing in paediatric inflammatory bowel disease to screen for primary sclerosing cholangitis: a diagnostic study based on routinely collected electronic healthcare data.Archives of disease in childhood · 2025Article
- Gamma-glutamyl transferase and disease course in pediatric-onset primary sclerosing cholangitis: A single-center cohort study.Health science reports · 2023Article
- Biological synthesis of ursodeoxycholic acid.Frontiers in microbiology · 2023Review
- The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) Index: A Prognostic Tool for Children.Hepatology (Baltimore, Md.) · 2021Article
- Pediatric Cholestatic Liver Disease: Review of Bile Acid Metabolism and Discussion of Current and Emerging Therapies.Frontiers in medicine · 2020Review
- A Prospective Trial of Withdrawal and Reinstitution of Ursodeoxycholic Acid in Pediatric Primary Sclerosing Cholangitis.Hepatology communications · 2019Article
- A Current Understanding of Bile Acids in Chronic Liver Disease.Journal of clinical and experimental hepatologyReview
- Diagnosis of Primary Sclerosing Cholangitis Beyond Childhood is Associated with Worse Outcomes.Journal of clinical and experimental hepatologyArticle
Corrections and comments
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Authors and funding
25 authors at 20 institutions in 7 countries.
Funding
Abstract
objectiveTo investigate patient factors predictive of gamma glutamyltransferase (GGT) normalization following ursodeoxycholic acid (UDCA) therapy in children with primary sclerosing cholangitis. STUDY
designWe retrospectively reviewed patient records at 46 centers. We included patients with a baseline serum GGT level ≥50 IU/L at diagnosis of primary sclerosing cholangitis who initiated UDCA therapy within 1 month and continued therapy for at least 1 year. We defined "normalization" as a GGT level <50 IU/L without experiencing portal hypertensive or dominant stricture events, liver transplantation, or death during the first year.
resultsWe identified 263 patients, median age 12.1 years at diagnosis, treated with UDCA at a median dose of 15 mg/kg/d. Normalization occurred in 46%. Patients with normalization had a lower prevalence of Crohn's disease, lower total bilirubin level, lower aspartate aminotransferase to platelet ratio index, greater platelet count, and greater serum albumin level at diagnosis. The 5-year survival with native liver was 99% in those patients who achieved normalization vs 77% in those who did not.
conclusionsLess than one-half of the patients treated with UDCA have a complete GGT normalization in the first year after diagnosis, but this subset of patients has a favorable 5-year outcome. Normalization is less likely in patients with a Crohn's disease phenotype or a laboratory profile suggestive of more advanced hepatobiliary fibrosis. Patients who do not achieve normalization could reasonably stop UDCA, as they are likely not receiving clinical benefit. Alternative treatments with improved efficacy are needed, particularly for patients with already-advanced disease.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.