In March 2023, the FDA extended the approval of Evinacumab (EVKEEZA©) to children 5-11 years with familial homozygous hypercholesteremia. Evinacumab had been previously approved for 12 and older (2021), as an add-on treatment in children and adolescents with familial homozygous hypercholesteremia. Evinacumab is a monoclonal antibody which blocks angioprotein-3 activity which targets hepatic and endothelial lipase and causes LDL-C reduction by enhancing VLDL-C clearance. Evinacumab is given as an infusion at 15 mg/kg over one hour every 4 weeks. The common adverse effects include dizziness, flu-like symptoms, nausea and pain in extremities. Evkeeza can potentially affect the fetus therefore appropriate counseling and monitoring are recommended.
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