Primary sclerosing cholangitis (PSC) is a rare,
progressive, cholestatic, immune-mediated hepatobiliary disease. Although 90%
of patients with PSC have inflammatory bowel disease (IBD), only 2.4-4% of
patients with ulcerative colitis and 1.4% of patients with Crohn’s disease have
PSC. The association between PSC and hepatobiliary malignancies and colorectal
cancer is well established. Diagnostic criteria are elevated serum alkaline
phosphatase levels persisting for more than 6 months, cholangiographic findings
of bile duct strictures detected by magnetic resonance cholangiopancreatography
(MRCP) or endoscopic retrograde cholangiopancreatography (ERCP) and exclusion
of secondary causes of sclerosing cholangitis. Liver transplantation is the
only proven effective treatment for patients with advanced PSC to date.
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