While homozygous mutations within ABCB4 may lead to PFIC type 3, patients with a mild phenotype or who are heterozygous for mutations in ABCB4 have been found in increasing numbers in several cholestatic conditions of adulthood including low phospholipid-associated cholelithiasis syndrome (LPAC) and intrahepatic cholestasis of pregnancy (ICP) (23, 24). The gene discussed is ABCB4; the disease is gallbladder disease 1.