Adpkd Hepatobiliary Manifestations

Finding

Frequency

Asymptomatic liver cysts

Symptomatic polycystic liver disease Complicated cysts (hemorrhage, infection) Massive hepatomegaly Chronic pain/discomfort Early satiety Supine dyspnea Abdominal hernia Obstructive jaundice Hepatic venous outflow obstruction Congenital hepatic fibrosis Idiopathic dilatation of intrahepatic or extrahepatic biliary tract Cholangiocarcinoma

Very common; increased prevalence with age (up to 80% at age 60) Uncommon (male/female ratio: 1/10)

Rare (not dominantly transmitted) Very rare

Very rare

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