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Primary Biliary Cirrhosis (PBC) is an idiopathic, cholestatic destruction of intra-
hepatic microscopic bile ducts. Up to 80% of the patients presenting with
PBC are females. Patients usually present with fatigue, pruritis and jaundice. Suspicion
of PBC arises when cholestasis and cirrhosis are present in middle-aged
women. Anti-mitochondrial antibodies (AMA) are present in 95% of the patients.
AMA is considered the serological hallmark of PBC, but may be absent in
a proportion of these patients. We hereby present a case of AMA-negative PBC.
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