PSC scientific research blog

Environmental Risk Factors of Pediatric-Onset Primary Sclerosing Cholangitis and Autoimmune Hepatitis.

9/5/2016

Abstract

OBJECTIVES: The aim of this population-based observational case-control questionnaire study was to investigate the possible role of environmental risk factors associated with pediatric-onset autoimmune liver diseases.
METHODS: Seventy-one patients with autoimmune liver diseases (<16 years) received a questionnaire with 22 items, evaluating contact with environmental factors (eg, family manners, type of housing, pets) before the diagnosis. Two age- and sex-matched control groups were used: inflammatory bowel disease (IBD; n = 91) and healthy subjects (n = 716; matched also for place of residence at birth . . . Please click here to read the remainder of the abstract.

Tenca A, et al.
J Pediatr Gastroenterol Nutr. 2016.

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A Review of the Challenges Associated with the Diagnosis and Therapy of Primary Sclerosing Cholangitis

9/4/2016

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Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic liver disease that often leads to the development of cirrhosis. Complications of PSC include pruritus, fatigue, vitamin deficiencies, metabolic bone disease, dominant biliary strictures, gallstones, and hepatobiliary malignancies, most commonly cholangiocarcinoma (CCA). Despite the presumed autoimmune etiology of PSC, a clear benefit from immunosuppressive agents has not yet been established, and their use is limited by their side effects. Endoscopy is required in evaluation of biliary strictures in PSC to rule out the possibility of CCA ... The full text of this article is available by clicking here.

Mohammed Saadi, Christine Yu, and Mohamed O. Othman
J Clin Transl Hepatol. 2014 Mar; 2(1): 45–52.
Published online 2014 Mar 15. doi: 10.14218/JCTH.2013.00021

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Clinical course and prognosis of pediatric-onset primary sclerosing cholangitis

9/4/2016

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Abstract:
Background: The natural history of pediatric-onset primary sclerosing cholangitis (PSC) and overlap with autoimmune hepatitis (PSC/AIH) is poorly known.
Objective: The aim of this study was to evaluate the clinical outcome of patients with pediatric-onset disease in a tertiary referral center.
Methods: We traced 33 patients (median age at diagnosis 16 years), with PSC or PSC/AIH in cholangiography and liver histology diagnosed between December 1993 and 2011, at Helsinki University Hospital. Diagnostic procedures and long-term follow-up were reassessed until the end of December 2013.
Results: PSC was confirmed in all 33 patients; 19 of them had an overlap with AIH. At diagnosis, three of 33 had cirrhosis. Inflammatory bowel disease (IBD) was associated in 76% of the patients, mostly ulcerative colitis (70%); treatment of IBD being a minor determinant of the clinical outcome of liver disease ... please click here for the remainder of the article which is freely available..

Andrea Tenca, Martti Fa ̈rkkila ̈, Johanna Arola, Tytti Jaakkola, Roberto Penagini and Kaija-Leena Kolho
United European Gastroenterology JournalDOI: 10.1177/2050640615616012

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Applicability and prognostic value of histologic scoring systems in primary sclerosing cholangitis

9/4/2016

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BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. At present, there is no appropriate histologic scoring system available for PSC, evaluating both degree of necroinflammatory activity (grade) and fibrosis (stage). The aim of this study was to assess if three scoring systems, commonly used in different liver diseases could be applied for grading and/or staging of PSC.

The remainder of the abstract can be found at this link.

Elisabeth M G de Vries, Joanne Verheij, Stefan G Hubscher, Mariska M G Leeflang, Kirsten Boonstra, Ulrich Beuers, Cyriel Y Ponsoen, Journal of Hepatology 2015, 63 (5): 1212-9

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Scientific Research
This page is in a'blog' format with the latest scientific PSC research identified by Google Scholar, Readbyqmx.com and other sources added regularly.

Environmental Risk Factors of Pediatric-Onset Primary Sclerosing Cholangitis and Autoimmune Hepatitis.

A Review of the Challenges Associated with the Diagnosis and Therapy of Primary Sclerosing Cholangitis

Clinical course and prognosis of pediatric-onset primary sclerosing cholangitis

Applicability and prognostic value of histologic scoring systems in primary sclerosing cholangitis

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