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ALIMENTARY TRACT AND METABOLISM › BILE AND LIVER THERAPY › BILE THERAPY › Bile acids and derivatives
chenodeoxycholic acid
A05AA01
Adult Dosing
- Initial dose: 250 mg orally twice daily (morning and night) for the first 2 weeks
- Titration: Increase by 250 mg/day every 2 weeks as tolerated to achieve target dose
- Maintenance dose: 12.5 to 15 mg/kg/day divided in 2 daily doses
- Maximum dose: Typically 250 mg/day for specific inherited bile acid synthesis disorders or up to 750-1000 mg/day based on weight
Pediatric Dosing
- Neonates, infants, children, and adolescents:
- Initial dose: 5 mg/kg/day divided into 2 or 3 doses
- Maintenance dose: 15 mg/kg/day divided into 2 or 3 doses
- Titration: Adjust based on clinical response and liver function tests
Indications
- Treatment of inborn errors of primary bile acid synthesis due to specific enzyme defects
- Treatment of cerebrotendinous xanthomatosis (CTX) (off-label/specialist use)
Mechanism of Action
- Primary bile acid that acts as a ligand for the farnesoid X receptor (FXR)
- Suppresses hepatic synthesis of cholesterol 7 alpha-hydroxylase (rate-limiting step in bile acid synthesis)
- Replaces deficient natural primary bile acids in inherited synthesis defects, reducing toxic intermediate metabolites and improving bile flow
Contraindications
- Complete biliary obstruction
- Active acute hepatocellular liver disease
- Known hypersensitivity to chenodeoxycholic acid or any excipients
Adverse Reactions
- Elevated liver function tests (ALT, AST, bilirubin)
- Diarrhea
- Abdominal pain
- Nausea
- Pruritus
- Fatigue
Drug Interactions
- Bile acid sequestrants (e.g., cholestyramine, colestipol) bind chenodeoxycholic acid in the gut, decreasing its absorption; separate administration by at least 1-2 hours
- Aluminum-based antacids may adsorb bile acids and reduce efficacy
- Hepatotoxic drugs may potentiate the risk of liver injury
- Estrogens, oral contraceptives, and clofibrate increase biliary cholesterol secretion and may counteract the therapeutic efficacy of chenodeoxycholic acid
Curated Content: Needs Vetting Before Put to Clinical Use