Homozygous familial hypercholesterolaemia

Active Ingredient: Evinacumab

Indication for Evinacumab

Population group: only children (1 year - 12 years old) , adolescents (12 years - 18 years old) , adults (18 years old or older)
Therapeutic intent: Curative procedure

Evinacumab is indicated as an adjunct to diet and other low-density lipoprotein-cholesterol (LDL-C) lowering therapies for the treatment of adult and paediatric patients aged 5 years and older with homozygous familial hypercholesterolaemia (HoFH).

For this indication, competent medicine agencies globally authorize below treatments:

15 mg/kg once monthly

For:

Dosage regimens

Intravenous, 15 milligrams evinacumab per kilogram of body weight, once monthly.

Detailed description

The recommended dose is 15 mg/kg administered by intravenous infusion over 60 minutes once monthly (every 4 weeks).

If a dose is missed, it should be administered as soon as possible. Thereafter, treatment with evinacumab should be scheduled monthly from the date of the last dose.

The rate of infusion may be slowed, interrupted or discontinued if the patient develops any signs of adverse reactions, including infusion-associated symptoms.

Evinacumab can be administered without regard to lipoprotein apheresis.

Dosage considerations

Evinacumab should be administered over 60 minutes by intravenous infusion. Do not administer evinacumab as an intravenous push or bolus.

The rate of infusion may be slowed, interrupted, or discontinued if the patient develops any signs of adverse reactions, including infusion-associated symptoms.

Active ingredient

Evinacumab

Evinacumab is a recombinant human monoclonal antibody, which specifically binds to and inhibits ANGPTL3. ANGPTL3 is a member of the angiopoietin-like protein family that is expressed primarily in the liver and plays a role in the regulation of lipid metabolism by inhibiting lipoprotein lipase (LPL) and endothelial lipase (EL). Evinacumab blockade of ANGPTL3 lowers TG and HDL-C by releasing LPL and EL activities from ANGPTL3 inhibition, respectively.

Read more about Evinacumab

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