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Duchenne Muscular DystrophyPhase 2NCT03167255

Extension Study of NS-065/NCNP-01 in Boys With Duchenne Muscular Dystrophy (DMD)

A Phase 2 study testing NS-065/NCNP-01 in people with Duchenne Muscular Dystrophy, run by NS Pharma, Inc. at 6 sites in 2 countries.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record
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Trial summary

This is an open-label, extension study of NS-065/NCNP-01 administered intravenously once weekly for an additional 192 weeks to boys with DMD who complete Study NS-065/NCNP-01-201. Read more about this study

Who is it for?
Ages 4 to 10 years, men only
Conditions the study is for
Duchenne Muscular Dystrophy
What is being tested?
NS-065/NCNP-01
Type of treatment
Injection or infusion
Could I receive a placebo?
No, everyone receives an active treatment
How many people
16 participants
Where
6 sitesin 2 countries
Study dates
6 Jul 2017to 15 Nov 2021
Phase
Phase 2
Are there results?
Yes, posted in the registrySummary in the doctors' view

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 4 to 10 years
  • Men only
  • No healthy volunteers
  • Child

Who may be able to join

  • Completed Study NS-065/NCNP-01-201 through Week 25.
  • Willing and able to comply with scheduled visits, investigational product administration plan, and study procedures.
  • Stable dose of glucocorticoid (GC), and is expected to remain on the stable dose for the duration of the study.

Who cannot join

  • Serious or severe adverse event in Study NS-065/NCNP-01-201 that precludes safe use of NS-065/NCNP-01.
  • Patient had a treatment which was made for the purpose of dystrophin or its related protein induction after completion of Study NS-065/NCNP-01-201.
  • Patient took any other investigational drugs after completion of Study NS-065/NCNP-01-201.
  • Patient was judged by the investigator and/or the Sponsor that it was not appropriate to participate in the extension study for other reasons.

Trial locations and contacts

6 sites in 2 countries.

6 sites in 2 countries

United States5 sites
  • Lurie Children's Hospital

    Chicago, Illinois, 60611

  • Duke University Medical Center

    Durham, North Carolina, 27710

  • Children's Hospital of Richmond at VCU

    Richmond, Virginia, 23298

  • UC Davis

    Sacramento, California, 95817

  • Washington University

    St Louis, Missouri, 63110

Other trials in United States

Canada1 site
  • Alberta Children's Hospital

    Calgary, Alberta

Other trials in Canada

About this study

Official title: A Phase II, Open-Label, Extension Study to Assess the Safety and Efficacy of NS-065/NCNP-01 in Boys With Duchenne Muscular Dystrophy (DMD)

This is an open-label, extension study of NS-065/NCNP-01 administered intravenously once weekly for an additional 192 weeks to boys with DMD who complete Study NS-065/NCNP-01-201.

Read the detailed description

This is a Phase II, multicenter, open-label, extension study of NS-065/NCNP-01 administered intravenously once weekly for an additional 192 weeks to boys with DMD who complete Study NS-065/NCNP-01-201. This study will evaluate the safety, tolerability, and clinical efficacy of NS-065/NCNP-01 at dose levels of up to 80 mg/kg/week administered by weekly IV infusion over an additional treatment period of 192 weeks or until enrollment in a separate long-term follow up program of NS-065/NCNP-01, whichever is earlier.

Patients who complete the Phase II Dose-finding Study NS-065/NCNP-01-201 are eligible to enroll.

Being tested
NS-065/NCNP-01
Medical subject headings
Muscular Dystrophy, Duchenne

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

  • NS-065/NCNP-01Drug

    Received during weekly intravenous infusions

Study groups in NCT03167255
Study groupRoleWhat they receive
NS-065/NCNP-01 40mg/kgExperimentalPatients receiving 40mg/kg in the NS-065-NCNP-201 study will continue their current dose for an additional 192 weeks or until enrollment in a separate long-term follow up program of NS-065/NCNP-01, whichever is earlier.
Receives: Drug: NS-065/NCNP-01
NS-065/NCNP-01 80mg/kgExperimentalPatients receiving 80mg/kg in the NS-065-NCNP-201 study will continue their current dose for an additional 192 weeks or until enrollment in a separate long-term follow up program of NS-065/NCNP-01, whichever is earlier.
Receives: Drug: NS-065/NCNP-01

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. Change From Baseline in Time to Stand (TTSTAND) Versus Matched Historical Controls

    A primary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Time to Stand (TTSTAND)

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

  2. Change From Baseline in Time to Stand (TTSTAND) Velocity Versus Matched Historical Controls

    A primary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Time to Stand (TTSTAND) Velocity

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

  3. Number of Participants With Treatment Related Adverse Events as Assessed by CTCAE v4.0.

    For adverse events (AEs) starting in study 201 (NCT02740972) which are not resolved at the time of enrollment into this study 202, any change in outcome or relatedness were reported in study 201.

    For AEs starting in study 201 which increase in severity or becomes serious after enrollment in this study 202, a new AE was reported in this study.

    Treatment-emergent AEs (TEAEs) were summarized by dose level. Coding was done by system organ class and preferred term (using the Medical Dictionary for Regulatory Activities (MedDRA)). Level of severity was assessed using the CTCAE grading system.

    Measured: Up to 192 weeks of treatment

Secondary outcomes (11)
  1. Change From Baseline in Time to Run/Walk 10 Meters Test (TTRW) Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Time to Run/Walk 10 meters test (TTRW)

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

  2. Change From Baseline in Time to Run/Walk 10 Meters Test (TTRW) Velocity Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Time to Run/Walk 10 meters test (TTRW) Velocity.

    The results were converted into velocity (meter/time).

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

  3. Change From Baseline in Time to Climb 4 Stairs (TTCLIMB) Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Time to Climb 4 stairs (TTCLIMB)

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

  4. Change From Baseline in Time to Climb 4 Stairs (TTCLIMB) Velocity Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Time to Climb 4 stairs (TTCLIMB) Velocity. The results were converted into velocity (meter/time).

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

  5. Change From Baseline in North Star Ambulatory Assessment (NSAA) Score Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): North Star Ambulatory Assessment (NSAA) score

    The NSAA is a functional scale devised for use in ambulant children with Duchenne muscular dystrophy (DMD).

    It consists of 17 activities graded 0 (unable to perform), 1 (performs with modifications), 2 (normal movement). It assesses abilities necessary to remain ambulant that have been found to progressively deteriorate in untreated DMD patients, as well as in other muscular dystrophies such as Becker Muscular Dystrophy. NSAA Total Score ranges from 0 to 34, with a score of 34 implying normal function.

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157 in Study 202

  6. Change From Baseline in Six-Minute Walk Test (6MWT) Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Six-Minute Walk Test (6MWT)

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157 in Study 202

  7. Change From Baseline in Quantitative Muscle Testing (QMT) for Handgrip Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Quantitative Muscle Testing (QMT) for Handgrip For QMT tests, the higher of each of the bilateral scores recorded for each muscle group at each visit were analyzed. QMT tests were analyzed by dominant/non-dominant side.

    QMT is a well-established method for measuring muscle weakness in neuromuscular disease. Patients will be placed on an examination table with a back-support system to eliminate the need for manual back stabilization. Following a single practice administration, each patient will complete a scored QMT evaluation (perform 2 tests; with the higher of the 2 values used for data analysis). QMT will be performed by recording force in pounds through a direct computer interface with a strain gauge.

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

  8. Change From Baseline in Quantitative Muscle Testing (QMT) for Elbow Flexors Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Quantitative Muscle Testing (QMT) for Elbow Flexors For QMT tests, the higher of each of the bilateral scores recorded for each muscle group at each visit were analyzed. QMT tests were analyzed by dominant/non-dominant side.

    QMT is a well-established method for measuring muscle weakness in neuromuscular disease. Patients will be placed on an examination table with a back-support system to eliminate the need for manual back stabilization. Following a single practice administration, each patient will complete a scored QMT evaluation (perform 2 tests; with the higher of the 2 values used for data analysis). QMT will be performed by recording force in pounds through a direct computer interface with a strain gauge.

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

  9. Change From Baseline in Quantitative Muscle Testing (QMT) for Elbow Extensors Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Quantitative Muscle Testing (QMT) for Elbow Extensors For QMT tests, the higher of each of the bilateral scores recorded for each muscle group at each visit were analyzed. QMT tests were analyzed by dominant/non-dominant side.

    QMT is a well-established method for measuring muscle weakness in neuromuscular disease. Patients will be placed on an examination table with a back-support system to eliminate the need for manual back stabilization. Following a single practice administration, each patient will complete a scored QMT evaluation (perform 2 tests; with the higher of the 2 values used for data analysis). QMT will be performed by recording force in pounds through a direct computer interface with a strain gauge.

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

  10. Change From Baseline in Quantitative Muscle Testing (QMT) for Knee Flexors Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Quantitative Muscle Testing (QMT) for Knee Flexors For QMT tests, the higher of each of the bilateral scores recorded for each muscle group at each visit were analyzed. QMT tests were analyzed by dominant/non-dominant side.

    QMT is a well-established method for measuring muscle weakness in neuromuscular disease. Patients will be placed on an examination table with a back-support system to eliminate the need for manual back stabilization. Following a single practice administration, each patient will complete a scored QMT evaluation (perform 2 tests; with the higher of the 2 values used for data analysis). QMT will be performed by recording force in pounds through a direct computer interface with a strain gauge.

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

  11. Change From Baseline in Quantitative Muscle Testing (QMT) for Knee Extensors Versus Matched Historical Controls

    A secondary efficacy endpoint was compared to Baseline of Study 201 (NCT02740972): Quantitative Muscle Testing (QMT) for Knee Extensors For QMT tests, the higher of each of the bilateral scores recorded for each muscle group at each visit were analyzed. QMT tests were analyzed by dominant/non-dominant side.

    QMT is a well-established method for measuring muscle weakness in neuromuscular disease. Patients will be placed on an examination table with a back-support system to eliminate the need for manual back stabilization. Following a single practice administration, each patient will complete a scored QMT evaluation (perform 2 tests; with the higher of the 2 values used for data analysis). QMT will be performed by recording force in pounds through a direct computer interface with a strain gauge.

    Measured: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted25 May 2017
  2. Study start6 Jul 2017
  3. Primary completion20 Oct 2021
  4. Study completion15 Nov 2021

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Interventional
Phase
Phase 2
Primary purpose
Treatment
Allocation
Non-randomised
Intervention model
Parallel groups
Model description
Low Dose cohort of 40 mg/kg and High Dose cohort of 80 mg/kg
Masking
None (open label)
Enrolment
16 participants (actual)
IDs, dates and publications
ClinicalTrials.gov ID
NCT03167255
Sponsor's protocol ID
NS-065/NCNP-01-202
Status last verified
Dec 2022
First submitted
22 May 2017
Last updated
28 Dec 2022
Data monitoring committee
Yes
FDA-regulated drug
Yes
FDA-regulated device
No
Individual data sharing
No

Questions about this trial

What is this clinical trial studying?

A Phase 2 study testing NS-065/NCNP-01 in people with Duchenne Muscular Dystrophy, run by NS Pharma, Inc. at 6 sites in 2 countries.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 3 inclusion and 4 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: finished, as of the sponsor's last update on 28 Dec 2022. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 6 sites in United States, Canada. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

What does this phase mean?

Phase 2 studies give the treatment to a larger group to see whether it has the intended effect and to keep checking safety.

Not every study follows the same path, and some studies (observational studies, device studies) have no phase.

Are results available for this study?

Yes. Results are posted in the registry (first posted 18 Nov 2022). A summary is in the For doctors view, and the full results are on ClinicalTrials.gov.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Change From Baseline in Time to Stand (TTSTAND) Versus Matched Historical Controls (time frame: Baseline 201, Weeks 37, 49, 73, 109, 157, 205 in Study 202). The study lists 3 primary outcomes in all. It is what the study was designed to measure, not a finding.

Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 28 Dec 2022.

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