Initial treatment is supportive, with the use of agents to treat<unk>  and pruritus, including the following: * Ursodeoxycholic acid * Cholestyramine * Rifampin * Naloxone, in refractory cases The partial external biliary diversion (PEBD) procedure is a surgical approach that diverts bile from the gallbladder externally into an ileostomy bag. Patients should be supplemented with fat-soluble vitamins, and occasionally medium-chain triglycerides in order to improve growth. When liver synthetic dysfunction is significant, patients should be listed for transplantation. Family members should be tested for<unk>  mutations, in order to determine risk of transmission.
Progressive familial intrahepatic cholestasis
Vanishing bile duct syndrome
Autoimmune hepatitis
Congenital hepatic fibrosis
Intrahepatic cholestasis of pregnancy
Familial cirrhosis
Alcoholic hepatitis
Neonatal cholestasis