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October 20, 2023CirculationOpen Access

Evinacumab for Pediatric Patients With Homozygous Familial Hypercholesterolemia

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Why the study?

Severely elevated LDL-C in HoFH starts in utero and accelerates atherosclerosis, creating an unmet need for aggressive LDLR-independent lipid-lowering therapies in pediatric patients.

Does intravenous evinacumab reduce LDL-C in pediatric patients with homozygous familial hypercholesterolemia inadequately controlled on optimized lipid-lowering therapy?

Population

14 patients 5 to 11 years of age with HoFH and LDL-C >130 mg/dL

Comparison

Intravenous evinacumab 15 mg/kg every 4 weeks

Design

Phase 3 open-label study

Follow-up

24 weeks

Authors

AWAlbert WiegmanSGSusanne Greber‐PlatzerSAShazia Ali

Discussion

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Member takes

Overview

May support LDL-C lowering with evinacumab in young HoFH patients; leaves open need for randomized outcome trials.

Structured PICO

Does intravenous evinacumab reduce LDL-C in pediatric patients with homozygous familial hypercholesterolemia inadequately controlled on optimized lipid-lowering therapy?

P
Population
14 pediatric patients 5 to 11 years of age with genetically proven homozygous familial hypercholesterolemia (HoFH; true homozygotes and compound heterozygotes) with LDL-C >130 mg/dL despite optimized lipid-lowering therapy (including LDLR-independent apheresis and lomitapide).
I
Intervention
Intravenous evinacumab 15 mg/kg every 4 weeks.
O
Outcome
LDL-C reduction from baseline to week 24surrogate

Evinacumab safely and effectively reduces LDL-C by nearly half in pediatric patients aged 5 to 11 years with homozygous familial hypercholesterolemia who are inadequately controlled on optimized lipid-lowering therapy.

Cite This Study

Wiegman et al. (2023) studied this question.

synapsesocial.com/papers/69ffcbecd1d8b50f8e9a0fechttps://doi.org/10.1161/circulationaha.123.065529
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