label: congenital liver fibrosis, PKHD1-related, horse
label_lc: congenital liver fibrosis, pkhd1-related, horse
description: "A developmental disorder of the biliary system caused by a congenital defect in the remodeling of the ductal plate at the level of the interlobular ducts, resulting in persistence of excessive abnormal embryonic bile duct structures, irregular portal veins, and progressive fibrosis of the portal tracts that occurs in horses due to a mutation in the PKHD1 gene."