Inflammation and progressive birosis and obstruction of the bile ducts.
Presents at 2 to 6 weeks after birth.
Probably not congental. ?viral (rota virus can cause scarring of bile ducts)
10% correctable (patent extrahepatic ducts).
90% palliate with Kasai operation (portoenterostomy) within 6/52 of birth.
2/3 will need a liver transplant.