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ALIMENTARY TRACT AND METABOLISM › BILE AND LIVER THERAPY › BILE THERAPY › Other drugs for bile therapy
maralixibat chloride
A05AX04
Adult Dosing
- Alagille syndrome: 38 mcg/kg once daily in the morning, may increase up to 57 mcg/kg once daily if needed
- Progressive familial intrahepatic cholestasis (PFIC): 57 mcg/kg once daily, may increase up to 142 mcg/kg once daily
Pediatric Dosing
- Patients aged 3 months and older: Same weight-based dosing as adults (ALGS: 38 to 57 mcg/kg daily; PFIC: 57 to 142 mcg/kg daily)
Indications
- Treatment of cholestatic pruritus in patients with Alagille syndrome (ALGS) aged 3 months and older
- Treatment of cholestatic pruritus in patients with progressive familial intrahepatic cholestasis (PFIC) aged 3 months and older
Mechanism of Action
- Reversible inhibition of the apical sodium-dependent bile acid transporter (ASBT)
- Decreases reabsorption of bile acids from the terminal ileum, increasing bile acid excretion in feces and lowering serum bile acid concentrations
Contraindications
- Complete biliary obstruction
- Hypersensitivity to maralixibat or any of its components
Adverse Reactions
- Diarrhea
- Abdominal pain
- Vomiting
- Fat-soluble vitamin deficiency
- Elevated aminotransferases
Drug Interactions
- OATP1B1 and OATP1B3 substrates: Potential for increased systemic exposure of concomitantly administered substrates
- Bile acid sequestrants (e.g., cholestyramine, colesevelam): May bind maralixibat and reduce its efficacy; administer at least 4 hours before or 4 hours after bile acid sequestrants
Curated Content: Needs Vetting Before Put to Clinical Use