An inherited disorder characterized by a combination of lymphoedema and intrahepatic cholestasis is described in a Norwegian kindred. The jaundice is evident soon after birth, and recurrent episodes occur throughout life. The oedema starts at about school age and subsequently progresses; it is due to hypoplasia of the lymph vessels of the lower extremities. The cause of the cholestasis has not been established, but a structural intrahepatic developmental defect is suggested.
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