Over 70% of patients are men, the majority of which are less than 45 years of age.
Presentation is usually with progressive fatigue, pruritus and jaundice.
Other symptoms are those associated with chronic liver disease, for example, weight loss, ascites, variceal bleeding and hepatic encephalopathy.
Hepatomegaly is found in about 50% of patients; less often, there is splenomegaly, hyperpigmentation and xanthomas.
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