Respuesta :
Inflammation of the intrahepatic and extrahepatic bile ducts is called Primary Sclerosing Cholangitis (PSC).
PSC is a rare condition of Cholangitis which exhibits fibrosis and inflammation of the intrahepatic as well as the extrahepatic biliary ductal systems which finally leads to biliary cirrhosis.
The bile duct system carries bile from the liver and gallbladder into the the duodenum which is identified as the first part of the small intestine.
Most patients are asymptomatic at diagnosis. It is associated with inflammatory bowel disease (mostly ulcerative colitis) and other autoimmune conditions. Elevated alkaline phosphatase is typical.
Diagnosis is made using imaging techniques such as Magnetic Resonance Cholangiopancreatography / Endoscopic Retrograde Cholangiopancreatography (MRCP/ERCP) or Ultrasonography which reveal an irregular beaded appearance of the biliary tract suggestive of multifocal strictures with associated segmental dilatations. Definitive treatment is liver transplantation.
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