Adult Primary Sclerosing Cholangitis (PSC) subjects have worse biliary disease at diagnosis compared to pediatric PSC subjects.

Autor: Kulkarni S; Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA. Electronic address: Sakil.Kulkarni@wustl.edu., Bhimaniya S; Department of Radiology, Washington University School of Medicine, St. Louis, MO, USA., Chi L; Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA., Tica S; Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA., Alghamdi S; Liver and Small Bowel Health Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia., Stoll J; Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA., Caudill K; Department of Radiology, Washington University School of Medicine, St. Louis, MO, USA., Fleckenstein J; Department of Internal Medicine, Washington University School of Medicine, St. Louis, MO, USA.
Jazyk: angličtina
Zdroj: Clinical imaging [Clin Imaging] 2023 May; Vol. 97, pp. 7-13. Date of Electronic Publication: 2023 Feb 04.
DOI: 10.1016/j.clinimag.2023.01.012
Abstrakt: Introduction: Adult Primary Sclerosing Cholangitis (PSC) subjects have worse outcomes compared to pediatric PSC subjects. The reasons for this observation are not completely understood.
Methods: In this single-center, retrospective (2005-17) study we compared clinical information, laboratory data, and previously published MRCP-based scores between 25 pediatric (0-18 years at diagnosis) and 45 adult (19 years and above) subjects with large duct PSC at the time of diagnosis. For each subject, radiologists determined MRCP-based parameters and scores after reviewing the MRCP images.
Results: The median age at diagnosis for pediatric subjects was 14 years, while that of adult subjects was 39 years. At the time of diagnosis, adult subjects had a higher incidence of biliary complications like cholangitis and high-grade biliary stricture (27% vs. 6%, p = 0.003) and higher serum bilirubin (0.8 vs. 0.4 mg/dl, p = 0.01). MRCP analysis showed that adult subjects had a higher incidence of hilar lymph node enlargement (24.4% vs. 4%, p = 0.03) at diagnosis. Adult subjects had worse sum-IHD score (p = 0.003) and average-IHD score (p = 0.03). Age at diagnosis correlated with higher average-IHD (p = 0.002) and sum-IHD (p = 0.002) scores. Adult subjects had worse Anali score without contrast (p = 0.01) at diagnosis. MRCP-based extrahepatic duct parameters and scores were similar between groups.
Discussion: Adult PSC subjects may have higher severity of disease at diagnosis compared to pediatric subjects. Future prospective cohort studies are required to confirm this hypothesis.
Competing Interests: Declaration of competing interest The authors do not have any financial or non-financial interests that are directly or indirectly related to the work submitted for publication.
(Copyright © 2023 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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