Aagenaes syndrome
Aagenaes syndrome, or lymphedema cholestasis syndrome (LSC1), is a form of idiopathic familial intrahepatic cholestasis associated with lymphedema of the lower extremities. Some cases of azine syndrome are said to be caused by mutations in the LS1 genome on chromosome 15q. This condition is inherited in an autosomal recessive fashion and is found primarily in people of Norwegian descent. Source: Genetic and Rare Diseases Information Center (GARD), supported by ORDR-NCATS and NHGRI. Neonatal cholestasis usually decreases in childhood in patients with azine syndrome, but it is intermittent in nature.

However,Aagenaes syndrome often gradually develops into cirrhosis of the liver and giant cell hepatitis, along with scarring of the portal tract tissue.