Version June 2024
| Medication | Category | Dose* | Adverse effects |
| First-line options | |||
| Ursodeoxycholic acid | Choleretic | 10 to 20 mg/kg/day orally, divided in 2 doses (maximum 300 mg twice daily) An oral liquid formulation is not commercially available but may be prepared by a compounding pharmacy (usual concentration 60 mg/mL) | Diarrhea, abdominal pain, worsening pruritus, or cholestasis at high dose |
| Diphenhydramine | Antihistamine | 5 mg/kg/day orally, divided in 3 or 4 doses (maximum 50 mg/dose) Oral liquid concentration – 2.5 mg/mL | Drowsiness, anticholinergic effects (eg, dry mouth, urinary hesitancy), impaired school performance |
| Hydroxyzine | Antihistamine | 2 mg/kg/day orally, divided in 3 or 4 doses (maximum 25 mg/dose) Oral liquid concentration – 2 mg/mL | |
| Rifampin (rifampicin) | Antibiotic | Patients ≥4 months – 4 to 10 mg/kg/day orally, divided in 2 doses (maximum 600 mg/day) An oral liquid formulation is not commercially available but may be prepared by a compounding pharmacy To deliver a dose of 150 or 300 mg, contents of a capsule may be mixed with applesauce, pudding, or other semisoft food | Red discoloration of body fluids, nausea, decreased appetite, hypersensitivity reactions, hepatitis, and altered metabolism of other drugs via induction of cytochrome P450 3A |
| Second-line options | |||
| Maralixibat¶ | Ileal bile acid transport inhibitor | Patients ≥3 months – 190 micrograms/kg orally once daily for 7 days (maximum initial dose 14.25 mg/day) and then increased to 380 micrograms/kg daily (maximum dose 28.5 mg daily) Available as 9.5 mg/mL oral solution | Gastrointestinal symptoms, malabsorption (including fat-soluble vitamins), increased transaminases |
| OdevixibatΔ | Ileal bile acid transport inhibitor | Patients ≥12 months – 120 micrograms/kg taken orally once daily | Gastrointestinal symptoms, hematoma, and decreased weight |
| Naltrexone | Opioid antagonist | 0.25 to 0.5 mg/kg orally once daily (maximum 50 mg/dose); limited pediatric data An oral liquid formulation is not commercially available but may be prepared by a compounding pharmacy | Self-limited opioid withdrawal-like syndrome can occur in patients not receiving opioids; elevated transaminases Avoid use in patients receiving opioids (reverses analgesia, precipitates withdrawal) |
| Third-line options | |||
| Sertraline | Selective serotonin reuptake inhibitor | 1 to 4 mg/kg/day orally (maximum 100 mg/day) Oral concentrate – 20 mg/mL; measured dose must be further diluted with 4 ounces (120 mL) water, ginger ale, lemon/lime soda, lemonade, or orange juice | Behavior disorders, skin reactions, vomiting, transient arterial hypertension Do not use in patients with moderate or severe hepatic impairment |
| Cholestyramine | Bile-salt binding agent | 240 mg/kg/day orally divided in 3 doses (maximum 8 g/day) Measured dose of oral powder (resin) must be mixed with 60 to 180 mL of water or other noncarbonated beverage | Constipation, abdominal pain, hyperchloremic metabolic acidosis, malabsorption (including fat-soluble vitamins) Not to be taken at same time with many other medications, due to decreased absorption; refer to drug interactions program included with UpToDate |
| Colesevelam | Bile-salt binding agent | Limited pediatric data Adult dose – 625 mg orally once daily; can titrate up to maximum 3.75 g daily in 1 or 2 divided doses Available as granules for suspension (3.75 g per packet); measured dose must be mixed with 240 mL of water, fruit juice, or diet soda | |
| Other | |||
| Atorvastatin | Lipid-lowering agent | Children and adolescents 10 to 17 years – 10 mg once daily; limited pediatric data Oral tablets may be crushed Available as 10 mg/2.5 mL oral suspension | Myalgias, myopathy, increased transaminases (usually transient, rarely hepatotoxic) |
FDA: US Food and Drug Administration.
* Commonly available concentrations (mg/mL) for commercially prepared oral liquids are listed. Other concentrations may be available. Consult Lexicomp drug reference and product labeling to confirm concentration of product dispensed.
¶ Maralixibat is approved by the FDA for treatment of cholestatic pruritus in people with Alagille syndrome ≥3 months of age.
Δ Odevixibat is approved by the FDA for the treatment of cholestatic pruritus in people with Alagille syndrome ≥12 months of age.Additional data from:
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