maralixibat; lopixibat (Livmarli)

Indications

• treatment of cholestatic pruritus in patients with Alagille syndrome >= 1 year

Dosage

• 380 ug/kg once daily, taken 30 minutes before 1st meal of day
  • starting dose: 190 ug/kg orally once daily
  • increase to 380 ug/kg once daily after one week, as tolerated

Oral solution: 9.5 mg of maralixibat per mL.

Adverse effects

• most common (> 5%)
  • diarrhea, abdominal pain, vomiting, fat-soluble vitamin deficiency, liver function test abnormalities, gastrointestinal bleeding, bone fractures

Laboratory

• baseline & periodic liver function tests

Mechanism of action

• ileal bile acid transporter (IBAT) inhibitor

More general terms

• enzyme inhibitor
• gastrointestinal agent

Database

• PubChem: http://pubchem.ncbi.nlm.nih.gov/summary/summary.cgi?cid=9831643
• PubChem: http://pubchem.ncbi.nlm.nih.gov/summary/summary.cgi?cid=9831642