ABCB11 and progressive familial intrahepatic cholestasis type 2: It was recently suggested that progressive familial intrahepatic cholestasis type 2 (PFIC 2), associated with a mutation of the ABCB11 gene resulting in deficiency of bile salt export pump (BSEP; a membrane canalicular bile acid transporter), represents a specific and previously unrecognized risk for HCC in young children [92].