The combination of neonatal intrahepatic cholestasis and lymphoedema in feet and 
legs is a specific syndrome named after the Norwegian paediatrician Øystein 
Aagenaes, who described the syndrome in 1968. The condition is autosomal 
recessively inherited and the gene is located to 15q, but not yet identified. 
The condition is particularly frequent in the southern most part of Norway and 
the gene frequency is estimated to be about 3%. The development of small 
lymphoid vessels is probably deficient around the small biliary tracts and in 
general. Aagenaes' syndrome is found in patients from other parts of Europe and 
the US, but more than half of the cases are of Norwegian origin.