Hereditary intrahepatic cholestasis with lymph oedema is now a well defined 
autosomal recessive inherited syndrome. More than 75% of the known cases (about 
40) are Norwegian, and most of these came from a few communities in the 
south-western part of Norway. Cholestasis is present prior to or shortly after 
birth. With modern treatment the cholestasis usually improves considerably 
during the first two years of life, but periods of recurrent cholestasis occur 
later. In some cases, lymph oedema is present at birth, but usually comes to 
light during childhood. Lymph oedema needs continuous treatment. As a rule, the 
prognosis for the liver disease is good, but cirrhosis has developed in about 
15% of the Norwegian cases. As for the pathogenesis of the cholestasis, the 
hypothesis is that the cause is an anomaly of the lymph function.