Clinical features

The majority of patients are between 10 and 30 years of age. Seventy-five percent are female.

Presentation is usually insidious. The patient may be generally unwell and jaundiced. Amenorrhoea is common. Epistaxis, bleeding gums and easy bruising may be other complaints.

Examination may reveal:

• spider naevi are almost invariable - usually on the face, neck or arms
• striae on the abdominal wall, usually the lateral aspect
• acne and hirsutism
• splenomegaly - in the absence of portal hypertension
• lymphadenopathy
• hepatomegaly - in early disease; the liver gradually shrinks
• ascites, oedema, hepatic encephalopathy - late features

Twenty-five percent of cases present as an acute hepatitis; About 20% of patients run an anicteric course.