As well as PFIC3, there is evidence that either biallelic or monoallelic ABCB4 defects may cause or predispose patients to a wide spectrum of human liver diseases, such as Low Phospholipid-Associated Cholelithiasis Syndrome (LPAC, OMIM #600803), Intrahepatic Cholestasis of Pregnancy (ICP, OMIM #147480), drug-induced liver injury, transient neonatal cholestasis, small duct sclerosing cholangitis, and adult biliary fibrosis or cirrhosis [58–61]. This evidence concerns the gene ABCB4 and Intrahepatic cholestasis of pregnancy.