Primary sclerosing cholangitis during childhood and adolescence

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Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic inflammatory disorder of the biliary system that results in progressive fibrosis and strictures of the intrahepatic and extrahepatic bile ducts. Typical radiological changes on retrograde cholangiography are shown in Figure 1. These strictures cause cholestatic liver disease that may progress to end-stage cirrhosis. The disorder has a strong ass...

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Primary Sclerosing Cholangitis: Introduction

The prevalence of primary sclerosing cholangitis in the United States is approximately 1–6 cases per 100,000 population. Most patients with primary sclerosing cholangitis are men (75%) with an average age of approximately 40 years at diagnosis. The overwhelming majority of patients affected with primary sclerosing cholangitis are Caucasian. The etiology is unknown but current opinion favors an ...

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Primary sclerosing cholangitis.

PRIMARY sclerosing cholangitis, stenosing cholangitis, fibrosing cholangitis, chronic obliterative cholangitis are all terms referring to a rare diffuse type ofchronic inflammation of the entire common bile duct and at times of the hepatic ducts and their radicles. In most instances, sclerosing cholangitis is idiopathic or primary. Bartholomew et al., in 1963 found only twenty unequivocal cases...

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Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a fibrosing disease of the intra- and extra-hepatic bile ducts, and is closely associated with inflammatory bowel disease. It is immune mediated, rather than being a classical autoimmune disease. A range of immune abnormalities have been demonstrated in PSC, in particular the findings of a range of autoantibodies, a portal tract infiltrate of functional T...

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Primary sclerosing cholangitis.

Primary sclerosing cholangitis (PSC) is an uncommon disorder of unknown etiology, characterized by chronic inflammation and fibrosis of the intra- and extrahepatic bile ducts. PSC is commonly associated with chronic inflammatory bowel disease, especially ulcerative colitis, and often in younger men with an extensive colitis. The diagnosis is made by endoscopic retrograde cholangiography. The ch...

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ژورنال

عنوان ژورنال: Clinical Liver Disease

سال: 2013

ISSN: 2046-2484

DOI: 10.1002/cld.251