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Two part (double-blind) inclisiran versus placebo [Year 1] followed by open-label inclisiran [Year 2] randomized multicentre study to evaluate safety, tolerability, and efficacy of inclisiran in adolescents (12 to less than 18 years) with homozygous familial hypercholesterolemia and elevated LDL-cholesterol (ORION-13)

Phase 3
Recruiting
Conditions
elevated LDL-cholesterol
homozygous familial hypercholesterolemia
10007510
10057166
Registration Number
NL-OMON54036
Lead Sponsor
ovartis
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
Recruiting
Sex
Not specified
Target Recruitment
2
Inclusion Criteria

Homozygous Familial Hypercholesterolemia (HoFH) diagnosed by genetic
confirmation.
Fasting LDL-C > 130 mg/dL (3.4 mmol/L) at screening.
On maximally tolerated dose of statin (investigator*s discretion) with or
without other lipid-lowering therapy; stable for >= 30 days before screening.
Estimated glomerular filtration rate (eGFR) > 30 mL/min/1.73 m2 at screening.
Other inclusion criteria are noted in the protocol.

Exclusion Criteria

Documented evidence of a null (negative) mutation in both LDLR alleles.
Heterozygous familial hypercholesterolemia (HeFH).
Active liver disease.
Secondary hypercholesterolemia, e.g. hypothyroidism or nephrotic syndrome.
Major adverse cardiovascular events within 1 month prior to randomization.
Previous treatment with monoclonal antibodies directed towards PCSK9 (within 90
days of screening).
Treatment with mipomersen or lomitapide (within 5 months of screening).
Recent and/or planned use of other investigational medicinal products or
devices.

Study & Design

Study Type
Interventional
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
<p>The primary objective is to demonstrate superiority of inclisiran compared to<br /><br>placebo in reducing LDL-C [percent change] at Day 330 (Year 1) in adolescents<br /><br>(aged 12 to <18 years) with HoFH and elevated LDL-C</p><br>
Secondary Outcome Measures
NameTimeMethod
<p> - Demonstrate superiority of inclisiran compared to placebo in reducing LDL-C<br /><br>[time-adjusted percent change] over Year 1<br /><br>- Evaluate the effect of inclisiran, compared to placebo (for Year 1) and<br /><br>long-term (up to Day 720), on lowering LDL-C, Apo B, lipoprotein (a) [Lp(a)],<br /><br>non-high density lipoprotein cholesterol (non-HDL-Cother lipoprotein and lipid<br /><br>parameters, and PCSK9 over time<br /><br>- Evaluate the safety and tolerability profile of inclisiran, compared to<br /><br>placebo (for Year 1) and long-term (up to Day 720), in adolescents (aged 12 to<br /><br><18 years) with HoFH </p><br>
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