Adult: In patients with Alagille syndrome: As 9.5 mg/mL oral solution: Initially, 190 mcg/kg once daily, then increase to the target dose of 380 mcg/kg once daily after one week. Max: 28.5 mg daily. Treatment recommendations may vary between countries and between individual products (refer to specific product guidelines). Child: ≥2 months Same as adult dose. Treatment recommendations may vary between countries and between individual products (refer to specific product guidelines).
Oral Cholestatic pruritus
Adult: In patients with progressive familial intrahepatic cholestasis: As 19 mg/mL oral solution: Initially, 285 mcg/kg once daily, then may increase incrementally to 285 mcg/kg bid, 428 mcg/kg bid, and then to the target dose of 570 mcg/kg bid as tolerated. Max: 38 mg daily. Treatment recommendations may vary between countries and between individual products (refer to specific product guidelines). Child: ≥12 months Same as adult dose. Treatment recommendations may vary between countries and between individual products (refer to specific product guidelines).
What are the brands available for Maralixibat in Hong Kong?
Previous or active hepatic decompensation events (e.g. variceal haemorrhage, ascites, hepatic encephalopathy). Contraindications may vary among countries and between individual products (refer to specific product guidelines).
Special Precautions
Patients with conditions that impair either gastrointestinal motility or enterohepatic circulation of bile acids; fat-soluble vitamin deficiency. Maralixibat is available in multiple concentrations and formulations approved for specific indications; these preparations are not interchangeable (refer to specific product and local guidelines prior to administration). Children. Pregnancy and lactation.
Adverse Reactions
Significant: Gastrointestinal effects (e.g. diarrhoea, nausea, vomiting, abdominal pain) that may lead to dehydration; fat-soluble vitamin (vitamins A, D, E, K) deficiency. Investigations: Increased INR, decreased Hb. Musculoskeletal and connective tissue disorders: Bone fracture. Potentially Fatal: Potential drug-induced liver injury, including decompensated liver disease or increased LFTs (e.g. elevated serum ALT, AST, GGT, bilirubin).
PO: Z (Avoid unless clinically required. Increased supplementation of fat-soluble vitamins may be needed if used during pregnancy.)
Monitoring Parameters
Monitor LFTs (at baseline, frequently for the 1st 6-8 months after starting therapy, then as clinically indicated); fat-soluble vitamin levels and INR (before treatment initiation and as clinically indicated). Assess for gastrointestinal symptoms (e.g. vomiting, diarrhoea); signs of hepatic decompensation and development of liver-related adverse reactions.
Drug Interactions
Concomitant administration with bile acid sequestrants (e.g. colestipol, colesevelam, colestyramine) may decrease the absorption and efficacy of maralixibat.
Action
Description: Mechanism of Action: Maralixibat, a reversible inhibitor of the ileal bile acid transporter (IBAT), reduces the reabsorption of bile acids from the terminal ileum. The mechanism of action by which it treats pruritus is unknown; however, it may be attributed to the inhibition of IBAT that leads to a subsequent decrease in serum bile acid levels. Pharmacokinetics: Absorption: Minimally absorbed. Bioavailability: approx <1%. Distribution: Plasma protein binding: 91%. Excretion: Mainly via faeces (73%; 94% as unchanged drug); urine (0.066%). Elimination half-life: 1.6 hours.
Chemical Structure
Maralixibat Source: National Center for Biotechnology Information. PubChem Compound Summary for CID 9831643, Maralixibat. https://pubchem.ncbi.nlm.nih.gov/compound/Maralixibat. Accessed June 26, 2025.
Storage
Store the intact bottle between 15-30°C. Once opened, store below 30°C and use within 130 days. Protect from light.
A05AX04 - maralixibat chloride ; Belongs to the class of other drugs used in bile therapy.
References
Brayfield A, Cadart C (eds). Maralixibat. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 02/06/2025.Joint Formulary Committee. Maralixibat. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 02/06/2025.Livmarli 9.5 mg/mL Oral Solution (Mirum Pharmaceuticals International BV). MHRA. https://products.mhra.gov.uk. Accessed 02/06/2025.Livmarli Oral Solution 9.5 mg/mL (CANbridge Care Pharma HongKong Limited). MIMS Hong Kong. http://www.mims.com/hongkong. Accessed 02/06/2025.Livmarli Solution and Tablet (Mirum Pharmaceuticals Inc.). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed. Accessed 02/06/2025.Maralixibat Chloride. UpToDate Lexidrug, AHFS DI (Adult and Pediatric) Online. American Society of Health-System Pharmacists, Inc. Waltham, MA. UpToDate, Inc. https://online.lexi.com. Accessed 02/06/2025.Maralixibat. UpToDate Lexidrug, Lexi-Drugs Multinational Online. Waltham, MA. UpToDate, Inc. https://online.lexi.com. Accessed 02/06/2025.Paediatric Formulary Committee. Maralixibat. BNF for Children [online]. London. BMJ Group, Pharmaceutical Press, and RCPCH Publications. https://www.medicinescomplete.com. Accessed 13/06/2025.
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