Primary Biliary Cirrhosis (PBC) is a relatively rare cholestatic liver disease. The
first case was described by Addison and Gull in 1851. The name PBC is generally
accepted, however in fact this is a misnomer since cirrhosis is found in a minority
of patients. PBC is one of the most frequent causes of the “vanishing bile duct
syndrome”, characterized by cholestasis due to destruction and progressive disappearance
of small intrahepatic bile ducts. Until a few decades ago no effective
medical treatment was available and the prognosis for patients with symptomatic
disease was poor, with reported survival less than 10 years. The pathogenesis of
PBC has still not been resolved, but nowadays an effective therapy is available and
this has significantly improved the prospects for patients with this condition.
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