Maralixibat Chloride (1 brand name, 2 dosage forms, 1 route)
Available dosage forms: SOLUTION, TABLET.
Routes of administration: ORAL.
Brand Names
- LIVMARLI
Contraindications
LIVMARLI is contraindicated in patients with prior or active hepatic decompensation events (e.g., variceal hemorrhage, ascites, hepatic encephalopathy) [see Warnings and Precautions (5.1)].
Adverse Reactions
The following clinically significant adverse reactions are described elsewhere in labeling: •Hepatotoxicity [see Warnings and Precautions (5.1)] •Gastrointestinal Adverse Reactions [see Warnings and Precautions (5.2)] •Fat Soluble Vitamin (FSV) Deficiency [see Warnings and Precautions (5.3)]
Mechanism of Action
Maralixibat is a reversible inhibitor of the ileal bile acid transporter (IBAT). It decreases the reabsorption of bile acids (primarily the salt forms) from the terminal ileum. Pruritus is a common symptom in patients with ALGS or PFIC and the pathophysiology of pruritus in patients with ALGS or PFIC is not completely understood. Although the complete mechanism by which maralixibat improves pruritus in ALGS or PFIC patients is unknown, it may involve inhibition of the IBAT, which results in decreased reuptake of bile salts, as observed by a decrease in serum bile acids [see Clinical Pharmacology (12.2)].
Overdosage
Single doses of maralixibat up to 500 mg, approximately 18-fold higher than the recommended dose, have been administered in healthy adults and were tolerated without a meaningful increase in adverse effects when compared to lower doses. If an overdose occurs, discontinue LIVMARLI, monitor the patient for any signs and symptoms and institute general supportive measures if needed. LIVMARLI contains propylene glycol as an excipient. In cases of suspected overdose, monitor for signs of propylene glycol toxicity, including hemolysis, hyperosmolarity with anion gap metabolic acidosis, acute kidney injury, and CNS toxicity. Discontinue LIVMARLI if propylene glycol toxicity is suspected.