Maralixibat 380 µg/mL Suppliers & Bulk Manufacturers

Available Forms: Oral Solution

Available Strengths: 380 µg/mL

Reference Brands: Livmarli (USA/EU)

Category: Medical Aid

Maralixibat (Livmarli) is used to treat progressive familial intrahepatic cholestasis (PFIC) in pediatric patients. It works by inhibiting the ileal bile acid transporter (IBAT), reducing bile acid reabsorption in the intestine, decreasing systemic bile acid levels, and improving liver function and pruritus (itching) associated with cholestatic liver disease.

Product Description:

Maralixibat blocks the ileal bile acid transporter, the protein that recovers bile acids from the end of the small intestine and returns them to the liver. Normally this recycling is highly efficient and almost nothing is lost. Blocking it sends bile acids into the colon to be excreted instead, which lowers the systemic bile acid load. In cholestatic liver disease, where bile cannot drain normally, that accumulated load causes both liver injury and the extreme itch that defines these conditions in children.

It is used for cholestatic pruritus in Alagille syndrome and in progressive familial intrahepatic cholestasis in children. The endpoint that matters is not a laboratory value but sleep: children with these conditions scratch until they bleed and cannot rest, and families are exhausted. Diarrhoea is the expected effect, since bile acids in the colon draw in water. Fat-soluble vitamin deficiency requires monitoring and supplementation, and transaminases are followed.

Sourcing notes. Originator orphan product, on patent, supplied as an oral solution dosed by weight with a specific dispensing syringe that must be quoted with it. Very small patient numbers in a handful of paediatric hepatology centres. Refrigeration requirements and in-use shelf life should be confirmed with the manufacturer per presentation.