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PRIMARY BILIARY CIRRHOSIS

Primary biliary cirrhosis is chronic, progressive, autoimmune, cholestatic liver disease more common in middle-aged women. It is characterized by destruction of small to medium bile ducts, leading to cholestasis and frequently, end-stage liver disease.
Diagnostic features are chronic biochemical cholestasis, presence of antimitochondrial antibodies and the characteristic liver biopsy findings.
At present, the diagnosis is most often made in an asymptomatic patient who presents with abnormal lab results on a routine checkup or as part of workup for an associated illness.


 

Surgical Intervention

Liver Transplantation 

  • Liver transplantation is the only life-saving procedure for patients w/ progressive primary biliary cirrhosis (PBC) & consequent liver failure
    • Increasing bilirubin & decreasing albumin levels & a prolonged prothrombin time are signs of disease progression
    • Model for End-stage Liver Disease (MELD) score of 315 & >7.8 from the updated Mayo Clinic Natural History Model for primary biliary cirrhosis (PBC)
  • Consider liver transplantation in refractory ascites, recurrent spontaneous bacterial peritonitis, recurrent variceal bleeding, hepatic coma, hepatocellular carcinoma or hepatorenal syndrome type I
    • Uncontrollable pruritus, chronic fatigue & severe osteoporosis may also be indications for liver transplantation
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