Study to Evaluate Safety, Tolerability and Efficacy of Inclisiran in Children With Homozygous Familial Hypercholesterolemia

NCT ID: NCT06597006

Last Updated: 2025-12-12

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE3

Total Enrollment

9 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-02-28

Study Completion Date

2029-04-15

Brief Summary

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This is a pivotal phase III study designed to evaluate safety, tolerability, and efficacy of inclisiran in children (aged 2 to \<12 years) with homozygous familial hypercholesterolemia (HoFH) and elevated low density lipoprotein cholesterol (LDLC).

Detailed Description

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This is a two-part (1 year double-blind inclisiran versus placebo / 1 year open-label inclisiran) multicenter study designed to evaluate safety, tolerability, and efficacy of inclisiran in children (aged 2 to \<12 years) with homozygous familial hypercholesterolemia (HoFH) and elevated low density lipoprotein cholesterol (LDL-C) on stable standard of care background lipid-lowering therapy.

Conditions

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Familial Hypercholesterolemia - Homozygous

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Parallel (Year 1) to single-group (Year 2)
Primary Study Purpose

TREATMENT

Blinding Strategy

DOUBLE

Participants Investigators
Masked (Year 1) to No Masking (Year 2)

Study Groups

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Inclisiran

Year 1 - inclisiran sodium subcutaneous injection (given at Days 1, 90, and 270) Day 360 only - placebo subcutaneous injection Year 2 - inclisiran sodium subcutaneous injection (given at Days 450 and 630)

Group Type EXPERIMENTAL

Inclisiran

Intervention Type DRUG

Inclisiran (inclisiran sodium 300 mg subcutaneous (s.c.) for participants with body weight ≥23 kg, inclisiran sodium 180 mg s.c. for participants with body weight \<23 kg to ≥16 kg, or inclisiran sodium 100 mg s.c. for participants with body weight \<16 kg. The dose level is based on the participant's body weight on Day 1 (for Part 1) and Day 360 (for Part 2), respectively.

Placebo

Year 1 - placebo subcutaneous injection (given at Days 1, 90 and 270) Year 2 - inclisiran sodium subcutaneous injection (given at Days 360, 450, and 630)

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

Sterile normal saline (0.9% sodium chloride in water for subcutaneous injection)

Interventions

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Inclisiran

Inclisiran (inclisiran sodium 300 mg subcutaneous (s.c.) for participants with body weight ≥23 kg, inclisiran sodium 180 mg s.c. for participants with body weight \<23 kg to ≥16 kg, or inclisiran sodium 100 mg s.c. for participants with body weight \<16 kg. The dose level is based on the participant's body weight on Day 1 (for Part 1) and Day 360 (for Part 2), respectively.

Intervention Type DRUG

Placebo

Sterile normal saline (0.9% sodium chloride in water for subcutaneous injection)

Intervention Type DRUG

Other Intervention Names

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KJX839 saline solution

Eligibility Criteria

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Inclusion Criteria

* Male or female participants, 2 to \<12 years of age at screening
* HoFH diagnosed by genetic confirmation

Exclusion Criteria

* Fasting LDL-C \>130 mg/dL (3.4 mmol/L) at screening
* On an optimal dose of statin (investigator's discretion), unless statin intolerant, with or without other lipid-lowering therapy (e.g. ezetimibe)
* Participants on lipid-lowering therapies (such as e.g. statins, ezetimibe) must be on a stable dose for ≥30 days before screening with no planned medication or dose changes during study participation
* Participants on a documented regimen of LDL-apheresis for ≥ 3 months before screening will be allowed to continue the apheresis during the study, if needed. The apheresis schedule/settings/duration must be stable prior to screening, are not allowed to change during the double-blind period of the trial and must permit that an apheresis coincides with each study visit.


* Documented evidence of a null (negative) mutation in both LDLR alleles
* Previous treatment (within 90 days of screening) with monoclonal antibodies directed towards PCSK9
* History of poor response to therapy with any monoclonal antibody directed towards PCSK9 (e.g. \<15% reduction in LDL-C)
* Treatment with mipomersen or lomitapide (within 5 months of screening)
* Secondary hypercholesterolemia, e.g. hypothyroidism or nephrotic syndrome
* Heterozygous familial hypercholesterolemia (HeFH)
* Body weight (at the screening and/or randomization (Day 1) visit) \<16 kg for participants 6 to \<12 years (at screening) or \<11 kg for participants 2 to \<6 years (at screening)
* Active liver disease defined as any known current infectious, neoplastic, or metabolic pathology of the liver or unexplained alanine aminotransferase (ALT), aspartate aminotransferase (AST) elevation \>3x ULN, or total bilirubin elevation \>2x ULN (except patients with Gilbert's syndrome)
* Pregnant or nursing females
* Recent and/or planned use of other investigational medicinal products or devices
Minimum Eligible Age

2 Years

Maximum Eligible Age

11 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Novartis Pharmaceuticals

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Novartis Pharmaceuticals

Role: STUDY_DIRECTOR

Novartis Pharmaceuticals

Locations

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UC San Francisco Medical Center

San Francisco, California, United States

Site Status RECRUITING

UC San Francisco Medical Center

San Francisco, California, United States

Site Status RECRUITING

Childrens National Hospital

Washington D.C., District of Columbia, United States

Site Status RECRUITING

Washington Univ School Of Medicine

St Louis, Missouri, United States

Site Status RECRUITING

Novartis Investigative Site

Vienna, , Austria

Site Status RECRUITING

Novartis Investigative Site

Beijing, Beijing Municipality, China

Site Status RECRUITING

Novartis Investigative Site

Frankfurt am Main, Hesse, Germany

Site Status RECRUITING

Novartis Investigative Site

Ioannina, , Greece

Site Status RECRUITING

Novartis Investigative Site

Kota Bharu, Kelantan, Malaysia

Site Status RECRUITING

Novartis Investigative Site

Amsterdam, North Holland, Netherlands

Site Status RECRUITING

Novartis Investigative Site

Bloemfontein, Free State, South Africa

Site Status RECRUITING

Novartis Investigative Site

Taichung, , Taiwan

Site Status RECRUITING

Novartis Investigative Site

Taipei, , Taiwan

Site Status RECRUITING

Novartis Investigative Site

Adana, Saricam, Turkey (Türkiye)

Site Status RECRUITING

Novartis Investigative Site

Ankara, Yenimahalle, Turkey (Türkiye)

Site Status RECRUITING

Novartis Investigative Site

Izmir, , Turkey (Türkiye)

Site Status RECRUITING

Novartis Investigative Site

Southampton, , United Kingdom

Site Status RECRUITING

Countries

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United States Austria China Germany Greece Malaysia Netherlands South Africa Taiwan Turkey (Türkiye) United Kingdom

Central Contacts

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Novartis Pharmaceuticals

Role: CONTACT

1-888-669-6682

Novartis Pharmaceuticals

Role: CONTACT

+41613241111

Facility Contacts

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Luis Gay

Role: primary

+1 415 990 7296

Luis Gay

Role: primary

+1 415 476 8338

Desiree Tobechukwu Nwanze

Role: primary

+1 202 476 5000

Jodi Pagano

Role: primary

Other Identifiers

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2024-514595-41

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

CKJX839C12304

Identifier Type: -

Identifier Source: org_study_id

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