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Krones, E; Graziadei, I; Trauner, M; Fickert, P.
Evolving concepts in primary sclerosing cholangitis.
Liver Int. 2012; 32(3):352-369 Doi: 10.1111/j.1478-3231.2011.02607.x [OPEN ACCESS]
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Leading authors Med Uni Graz
Tatscher Elisabeth
Trauner Michael
Co-authors Med Uni Graz
Fickert Peter
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Abstract:
Patients suffering from primary sclerosing cholangitis (PSC) show considerable differences regarding clinical manifestations (i.e. large duct versus small-duct PSC, presence or absence of concomitant inflammatory bowel disease), disease progression, risk for malignancy and response to therapy, raising the question whether PSC may represent a mixed bag of diseases of different aetiologies. The growing list of secondary causes and diseases 'mimicking' or even overlapping with PSC (e.g. IgG4-associated sclerosing cholangitis), which frequently causes problems in clear-cut discrimination from classic PSC and the emerging knowledge about potential disease modifier genes (e.g. variants of CFTR, TGR5 and MDR3) support such a conceptual view. In addition, PSC in children differs significantly from PSC in adults in several aspects resulting in distinct therapeutic concepts. From a clinical perspective, appropriate categorization and careful differential diagnosis are essential for the management of concerned patients. Therefore, the aim of the current review is to summarize current and evolving pathophysiological concepts and to provide up-to-date perspectives including future treatment strategies for PSC. © 2011 John Wiley & Sons A/S.
Find related publications in this database (using NLM MeSH Indexing)
Adult -
Age Factors -
Alkaline Phosphatase - blood
Biopsy -
Child -
Cholangiography - methods
Cholangitis, Sclerosing - classification Cholangitis, Sclerosing - complications Cholangitis, Sclerosing - diagnosis Cholangitis, Sclerosing - drug therapy Cholangitis, Sclerosing - epidemiology Cholangitis, Sclerosing - physiopathology
Diagnosis, Differential -
Humans -
Incidence -
Inflammatory Bowel Diseases - complications
Liver - pathology
Liver Transplantation - methods
Precancerous Conditions - pathology
Prevalence -

Find related publications in this database (Keywords)
autoimmune sclerosing cholangitis
children
IgG4-associated sclerosing cholangitis
inflammatory bowel diseases
primary sclerosing cholangitis
sclerosing cholangitis in critically ill patients
secondary sclerosing cholangitis
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