Liver pathology in PFIC-2. (A) Mild periportal inflammation and canalicular bile stasis seen in the liver biopsy of an 8-month old child; (B) ultrastructural demonstration of amorphous canalicular bile; (C) immunohistochemistry for Bile salt export protein (BSEP or ABCB11) shows complete lack of canalicular staining in the patient (C) as compared to the control (D) (immunochemistry performed by Alex Knisely, King’s College Hospital Liver Unit); (E) 8 years later, the patient presented with cirrhosis and cholestasis; (F) liver adenoma in the resected specimen. Magnification, 200× in A, C, D, 50× in E, and 40× in F. PFIC-2, progressive familial intrahepatic cholestasis-2.