Study to Assess the Efficacy and Safety of Viltolarsen in Ambulant Boys With DMD (RACER53)

NCT ID: NCT04060199

Last Updated: 2024-12-11

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

77 participants

Study Classification

INTERVENTIONAL

Study Start Date

2020-04-14

Study Completion Date

2023-10-19

Brief Summary

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The main objective of this study is to evaluate the efficacy of Viltolarsen compared to placebo in Duchenne muscular dystrophy (DMD) patients amenable to exon 53 skipping.

Detailed Description

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This is a Phase 3 randomized, double-blind, placebo-controlled, multi-center study to assess the efficacy and safety of Viltolarsen in ambulant boys with Duchenne muscular dystrophy. Eligible patients with out-of-frame deletion mutations amenable to exon 53 skipping will be randomized to receive once weekly intravenous (IV) infusions of 80 mg/kg Viltolarsen or placebo for up to 48 weeks.

The study will enroll approximately 74 patients amenable to exon 53 skipping. Clinical efficacy will be assessed at regularly scheduled study visits, including functional tests such as Time to Stand Test (TTSTAND), Time to Run/Walk 10 Meters Test (TTRW), Six-minute Walk Test (6MWT), North Star Ambulatory Assessment (NSAA), Time to Climb 4 Steps Test (TTCLIMB) and Hand-held dynamometer (elbow extension, elbow flexion, knee extension and knee flexion on the dominant side only).

Safety will be assessed through the collection of adverse events (AEs), laboratory tests, electrocardiograms (ECGs), vital signs, and physical examinations throughout the study.

Blood samples will be taken periodically throughout the study to assess the pharmacokinetics of study drug.

Conditions

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Duchenne Muscular Dystrophy

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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Viltolarsen

Patients amenable to exon 53 skipping will receive viltolarsen intravenous (IV) infusions, weekly, at 80 mg/kg for up to 48 weeks.

Group Type EXPERIMENTAL

Viltolarsen

Intervention Type DRUG

IV infusion

Placebo

Patients amenable to exon 53 skipping will receive placebo intravenous (IV) infusions, weekly, for up to 48 weeks.

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

IV infusion

Interventions

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Viltolarsen

IV infusion

Intervention Type DRUG

Placebo

IV infusion

Intervention Type DRUG

Other Intervention Names

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NS-065/NCNP-01

Eligibility Criteria

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Inclusion Criteria

* Male ≥ 4 years and \< 8 years of age
* Confirmed DMD mutation(s) in the dystrophin gene that is amenable to skipping of exon 53 to restore the dystrophin mRNA reading frame
* Able to walk independently without assistive devices
* TTSTAND \< 10 seconds
* Stable dose of glucocorticoid (GC) for at least 3 months prior to study entry and is expected to remain on stable dose of GC treatment for the duration of the study

Exclusion Criteria

* Current or history of chronic systemic fungal or viral infections
* Acute illness within 4 weeks prior to the first dose of study drug
* Evidence of symptomatic cardiomyopathy (Note: Asymptomatic cardiac abnormality on investigation would not be exclusionary)
* Allergy or hypersensitivity to the study drug or to any of its constituents
* Severe behavioral or cognitive problems that preclude participation in the study, in the opinion of the investigator
* Previous or ongoing medical condition, medical history, physical findings or laboratory abnormalities that could affect safety, make it unlikely that treatment and follow-up will be correctly completed or impair the assessment of study results, in the opinion of the investigator;
* Surgery within the 3 months prior to the first dose of study drug or surgery is planned for anytime during the duration of the study
* Participant has positive test results for hepatitis B antigen, hepatitis C antibody or human immunodeficiency virus (HIV)
* Currently taking any other investigational drug or has taken any other investigational drug within 3 months prior to the first dose of study drug or within 5 times the half-life of a medication, whichever is longer
* Previously enrolled in an interventional study of viltolarsen
* Currently taking any other exon skipping agent or has taken any other exon skipping agent within 3 months prior to the first dose of study drug
* Having taken any gene therapy
Minimum Eligible Age

4 Years

Maximum Eligible Age

7 Years

Eligible Sex

MALE

Accepts Healthy Volunteers

No

Sponsors

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Nippon Shinyaku Co., Ltd.

INDUSTRY

Sponsor Role collaborator

NS Pharma, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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University of California Davis Medical Center

Sacramento, California, United States

Site Status

Ann and Robert H. Lurie Children's Hospital of Chicago

Chicago, Illinois, United States

Site Status

Queensland Children's Hospital

Brisbane, , Australia

Site Status

Perth Children's Hospital

Nedlands, , Australia

Site Status

The Childrens Hospital at Westmead

Westmead, , Australia

Site Status

The Hospital for Sick Children (SickKids)

Toronto, Ontario, Canada

Site Status

Alberta Children's Hospital

Calgary, , Canada

Site Status

CHU de Quebec Research Centre

Québec, , Canada

Site Status

Hospital de Niños Roberto del Rio

Santiago, , Chile

Site Status

Pontificia Universidad Católica de Chile

Santiago, , Chile

Site Status

Chinese PLA General Hospital

Beijing, , China

Site Status

The Third Medical Center of PLA General Hospital

Beijing, , China

Site Status

Hunan Children's Hospital

Changsha, , China

Site Status

Children's Hospital of Fudan University

Shanghai, , China

Site Status

Shenzhen Children's Hospital

Shenzhen, , China

Site Status

Agia Sofia Children's Hospital

Athens, , Greece

Site Status

Hippokration General Hospital of Thessaloniki

Thessaloniki, , Greece

Site Status

Hong Kong Children's Hospital

Kowloon Bay, , Hong Kong

Site Status

Fondazione Policlinico Universitario A. Gemelli - Universita Cattolica del Sacro Cuore

Rome, , Italy

Site Status

Hospital Angeles Chihuahua

Chihuahua City, , Mexico

Site Status

Instituto Nacional de Pediatria

Mexico City, , Mexico

Site Status

Radboud Universitair Medisch Centrum

Nijmegen, Gelderland, Netherlands

Site Status

Leids Universitair Medisch Centrum

Leiden, , Netherlands

Site Status

New Zealand Clinical Research Ltd

Auckland, , New Zealand

Site Status

Rikshospitalet

Oslo, , Norway

Site Status

Russian National Research Medical University n.a. N.I.Pirogov, structural branch - Research Clinical Institute of Pediatrics n.a. Academician Yu. E. Veltishchev

Moscow, , Russia

Site Status

"Saint Petersburg State Paediatric Medical University" based at Consultative and Diagnostic Centre

Saint Petersburg, , Russia

Site Status

Tomsk National Research Medical Center of Russian Academy of Sciences

Tomsk, , Russia

Site Status

Pusan National University Yangsan Hospital

Pusan, , South Korea

Site Status

Seoul National University Hospital

Seoul, , South Korea

Site Status

Hospital Sant Joan de Deu

Barcelona, , Spain

Site Status

Hospital Universitario La Paz

Madrid, , Spain

Site Status

Kaohsiung Medical University Chung-Ho Memorial Hospital

Kaohsiung City, , Taiwan

Site Status

National Taiwan University Hospital

Taipei, , Taiwan

Site Status

Yeditepe University Kosuyolu Hospital

Istanbul, , Turkey (Türkiye)

Site Status

State Institution "Ukrainian Medical rehabilitation Center for Children with organic disorders of the nervous system of the Ministry of Health of Ukraine"

Kyiv, , Ukraine

Site Status

Birmingham Heartlands Hospital

Birmingham, , United Kingdom

Site Status

Royal Hospital for Children - Glasgow

Glasgow, , United Kingdom

Site Status

University College London Institute of Child Health

London, , United Kingdom

Site Status

Royal Manchester Children's Hospital

Manchester, , United Kingdom

Site Status

Countries

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Japan Sweden United States Australia Canada Chile China Greece Hong Kong Italy Mexico Netherlands New Zealand Norway Russia South Korea Spain Taiwan Turkey (Türkiye) Ukraine United Kingdom

Provided Documents

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Document Type: Study Protocol and Statistical Analysis Plan

View Document

Other Identifiers

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NS-065/NCNP-01-301

Identifier Type: -

Identifier Source: org_study_id