Systematic Review
Copyright ©The Author(s) 2019.
World J Hepatol. May 27, 2019; 11(5): 450-463
Published online May 27, 2019. doi: 10.4254/wjh.v11.i5.450
Table 1 Medical management of pruritus in children
MedicineDoseMechanism of action
CholestyramineInitial dose: 2 g BIDIon exchange resin which acts as BA binder in the intestine
(max dose 24 g/d)Decreased ileal BA absorption, Increased BA excretion (in feces)
NaltrexoneInitial dose: 0.25-5 mg/kg per dayOpioid antagonist
(max dose 50 mg/d)Block the permissive activity on pruritus neuronal signaling
RifampicinInitial dose: 5 mg/kgPXR agonist
Induces CYP3A4
(max dose 20 mg/kg per day)Increases metabolism and renal excretion of pruritogenic substances
Antibacterial effect may modify intestinal metabolism of pruritogenic substances
SertralineInitial dose: 1 mg/kg per daySerotonin reuptake inhibitor
(max dose: 4 mg/kg per day)Proposed mechanism includes increase in central serotonergic tone, which regulates pruritus
Ursodeoxycholic acid600 mg/m2 per dayTertiary BA
Increases bile secretion
Reduces ileal absorption of hydrophilic BAs
Table 2 Adult manifestations of progressive familial intrahepatic cholestasis gene mutations
EtiologyGenetic defectManifestations
FIC1 deficiencyATP8B1BRIC1
ICP1 and contraceptive-induced cholestasis
Cryptogenic cirrhosis
BSEP deficiencyABCB11BRIC2
ICP2 and contraceptive-induced cholestasis
Cryptogenic cirrhosis
MDR3 deficiencyABCB4ICP3 and contraceptive-induced cholestasis
Drug induced cholestasis
Low phospholipid-associated cholestasis
Cholesterol gallstone disease
Biliary fibrosis or liver cirrhosis without cholestasis
Cryptogenic cirrhosis
TJP2 deficiencyTJP2Cryptogenic cirrhosis
Drug induced cholestasis associated with propylthiouracil