Dosages
Side effects in children
Warnings & precautions in children
Contra-indications in children

Interactions
PK
Renal impairment
References

Maralixibat

Generic name
Maralixibat
Brand name
ATC Code
A05AX04

Pharmacokinetics in children

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dose recommendation of formulary compared to licensed use (on-label versus off-label)

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Available formulations

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Dosages

Progressive familial intrahepatic cholestasis (PFIC)
  • Oral
    • ≥ 3 months
      • Initial dose: 285 microg./kg/day in 1 dose
      • Maintenance dose: If needed Increase dose after 1-2 weeks based on effect and tolerance to 570 - 1.140 microg./kg/day in 2 doses. Max: 57 mg/day.
Cholestatic pruritus
  • Oral
    • ≥ 2 months
      • Initial dose: 190 microg./kg/day in 1 dose Increase initial dose after 1 week based on effect and tolerance to.
      • Maintenance dose: Increase initial dose after 1 week based on effect and tolerance to 380 microg./kg/day in 1 dose. Max: 28.5 mg/day.

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Renal impaiment in children > 3 months

Because of the propylene glycol content, the recommended maximum dose of Livmarli in patients with moderately impaired renal function (creatinine clearance CrCl ≥ 30 and < 60 ml/min) is 570 mcg/kg/dag. Livmarli should not be used in patients with severely impaired renal function.

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The complete list of all undesirable drug reactions can be found in the national Summary of Product Characteristics (SmPC) – click here

Side effects in children

The safety profile of maralixibat is consistent across all indications and age groups.

Very common (> 10%): abdominal pain and diarrhea (median duration < 1 week), diarrhea.
Common (1-10%): rise in ALAT and ASAT, in part with an increase in bilirubin.

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The complete list of all contra-indications can be found in the national Summary of Product Characteristics (SmPC) – click here

Contra-indications

No information available on specific contra indications in children.

The complete list of all warnings and precautions can be found in the national Summary of Product Characteristics (SmPC) – click here

Warnings & precautions in children

Check:

  • Before treatment: the values of fat-soluble vitamins (A, D and E) and of the INR. If these vitamins are deficient, additional therapy should be prescribed;
  • liver function values before and during treatment. Increases in ALAT (without increases in bilirubin) have been observed; their clinical significance is not known;
  • during episodes of diarrhea on adequate hydration.

Conditions, drugs or surgical procedures that affect gastrointestinal motility or enterohepatic circulation of bile acids may affect the efficacy of maralixibat.

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Interactions

The complete list of all interactions can be found in the national Summary of Product Characteristics (SmPC) – click here

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Bile acids and derivatives
A05AA02
Other drugs for bile therapy
A05AX05
BILE ACIDS AND DERIVATIVES
A05AA02

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Reference

  1. Mirum Pharmaceuticals International B.V., SmPC Livmarli (EU/1/22/1704/001) Rev 7; 12-7-2024, www.emea.europa.eu

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Changes

Changes