Expanding etiology of progressive familial intrahepatic cholestasis

Figure 1 Molecular mechanisms of cholestasis at the apical hepatocellular membrane. FXR: Farnesoid X receptor; RXR: Retinoid x receptor alpha; ABCB11: ATP binding cassette subfamily B member 11; MYO5B: Myosin VB; RAB11A: Ras-related protein Rab11a; FIC1: Familial intrahepatic cholestasis 1; BSEP: Bile salt export pump; MDR3: Multidrug resistance 3; TJP2: Tight junction protein 2.

Figure 2 Representative surgical interventions for progressive familial cholestasis. A: Partial external biliary diversion; B: Partial internal biliary diversion using a cholecystocolostomy approach; C: Ileal bypass/exclusion.