Long-Term Outcomes of Ataluren in Duchenne Muscular Dystrophy

NCT ID: NCT03179631

Last Updated: 2024-01-18

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

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

360 participants

Study Classification

INTERVENTIONAL

Study Start Date

2017-07-06

Study Completion Date

2023-07-25

Brief Summary

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This study is a long-term study of ataluren in participants with nonsense mutation Duchenne muscular dystrophy.

Detailed Description

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This study is a randomized, double-blind, placebo-controlled, 72-week study, followed by a 72-week open-label period. The purpose is to characterize the long-term effects of ataluren-mediated dystrophin restoration on disease progression. Participants will be randomized in a 1:1 ratio to ataluren or placebo. Participants will receive blinded study drug three times daily (TID) at morning, midday, and evening for 72 weeks, after which all participants will receive open-label ataluren for an additional 72 weeks (144 weeks in total). Study assessments will be performed at clinic visits every 12 weeks during the double-blind period and every 24 weeks during the open-label period. The total sample size of \~250 subjects will include \~160 subjects who meet the criteria for inclusion in the primary analysis population (age 7 to 16 years old, baseline six minute walk distance (6MWD) greater than or equal to (\>=) 300 meters, supine to stand \>= 5 seconds). The study will be conducted in the United States and other countries around the world.

Conditions

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Muscular Dystrophy, Duchenne Muscular Dystrophies Muscular Disorders, Atrophic Muscular Diseases Musculoskeletal Disease Neuromuscular Diseases Nervous System Diseases Genetic Diseases, X-Linked Genetic Diseases, Inborn

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

This study describes the randomized, double-blind, placebo-controlled, 72-week study and its 72-week open-label extension
Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors
A randomized, double-blind, placebo-controlled,72-week study and its 72-week open-label extension

Study Groups

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Ataluren

10, 20 milligrams per kilogram (mg/kg)

Group Type EXPERIMENTAL

Ataluren

Intervention Type DRUG

10, 20 mg/kg

Placebo

10, 20 mg/kg

Group Type PLACEBO_COMPARATOR

PLACEBO

Intervention Type DRUG

10, 20 mg/kg

Interventions

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Ataluren

10, 20 mg/kg

Intervention Type DRUG

PLACEBO

10, 20 mg/kg

Intervention Type DRUG

Other Intervention Names

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PTC124 Matching Placebo

Eligibility Criteria

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Inclusion Criteria

* Male sex
* Age ≥5 years
* Phenotypic evidence of Duchenne Muscular Dystrophy
* Nonsense point mutation in the dystrophin gene
* Use of systemic corticosteroids (prednisone/prednisolone or deflazacort)for a minimum of 12 months immediately prior to start of study treatment, with no significant change in dosage or dosing regimen for a minimum of 3 months immediately prior to start of study treatment
* 6MWD ≥150 meters
* Ability to perform timed function tests within 30 seconds
* Willingness and ability to comply with scheduled visits, drug administration plan, study procedures, laboratory tests, and study restrictions.

Exclusion Criteria

* Any change in prophylaxis treatment for cardiomyopathy within 1 month prior to start of study treatment.
* Ongoing intravenous (IV) aminoglycoside or IV vancomycin therapy.
* Prior or ongoing therapy with ataluren.
* Known hypersensitivity to any of the ingredients or excipients of the study drug
* Exposure to another investigational drug within 6 months prior to start of study treatment, or ongoing participation in any interventional clinical trial.
* History of major surgical procedure within 12 weeks prior to start of study treatment, or expectation of major surgical procedure during the 72-week placebo-controlled treatment period.
* Requirement for daytime ventilator assistance or any use of invasive mechanical ventilation via tracheostomy.
* Uncontrolled clinical symptoms and signs of congestive heart failure
* Elevated serum creatinine or cystatin C at screening.
Minimum Eligible Age

5 Years

Eligible Sex

MALE

Accepts Healthy Volunteers

No

Sponsors

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PTC Therapeutics

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Vinay Penematsa, MD

Role: STUDY_DIRECTOR

PTC Therapeutics, Inc.

Locations

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Phoenix Childrens Hospital

Phoenix, Arizona, United States

Site Status

Children's Hospital of Los Angeles

Los Angeles, California, United States

Site Status

University of California, San Francisco (UCSF) - Benioff Children's Hospital - Oakland

Oakland, California, United States

Site Status

Stanford University Medical Center

Palo Alto, California, United States

Site Status

University of California (UC) Davis Medical Center

Sacramento, California, United States

Site Status

Loma Linda University Children's Hospital

San Bernardino, California, United States

Site Status

Northwest Florida Clinical Research Group, LLC

Gulf Breeze, Florida, United States

Site Status

Indiana University Health - Riley Child Neurology

Indianapolis, Indiana, United States

Site Status

University of Kansas Medical Center

Kansas City, Kansas, United States

Site Status

University of Michigan - CS Mott Children's Hospital

Ann Arbor, Michigan, United States

Site Status

Children's Hospital of Michigan

Detroit, Michigan, United States

Site Status

University of Minnesota

Minneapolis, Minnesota, United States

Site Status

Columbia University College of Physicians & Surgeons

New York, New York, United States

Site Status

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status

Shriners Hospital for Children

Portland, Oregon, United States

Site Status

University of Pittsburgh School of Medicine

Pittsburgh, Pennsylvania, United States

Site Status

Cook Childrens Medical Center

Fort Worth, Texas, United States

Site Status

Texas Children's Hospital

Houston, Texas, United States

Site Status

University of Texas Health Science Center at San Antonio

San Antonio, Texas, United States

Site Status

University Of Utah

Salt Lake City, Utah, United States

Site Status

Children's Hospital of the King's Daughters

Norfolk, Virginia, United States

Site Status

Seattle Children's Hospital

Seattle, Washington, United States

Site Status

The Childrens Hospital at Westmead

Westmead, New South Wales, Australia

Site Status

The Royal Childrens Hospital

Parkville, Victoria, Australia

Site Status

Perth Children's Hospital

Nedlands, Western Australia, Australia

Site Status

Queensland Children's Hospital

South Brisbane, , Australia

Site Status

Hospital de Clínicas da Universidade Federal de Minas Gerais

Belo Horizonte, , Brazil

Site Status

Universidade Federal do Rio de Janeiro

Rio de Janeiro, , Brazil

Site Status

Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo - FMUSP

São Paulo, , Brazil

Site Status

UMHAT Sofiamed

Sofia, , Bulgaria

Site Status

Childrens Hospital London Health Sciences Centre

London, Ontario, Canada

Site Status

Childrens Hospital of Eastern Ontario

Ottawa, Ontario, Canada

Site Status

General Hospital of Chinese Armed Police Forces

Beijing, , China

Site Status

The First Affiliated Hospital of Fujian Medical University

Fuzhou, , China

Site Status

Xiangya Hospital Central South University

Hunan, , China

Site Status

Children's Hospital of Fudan University

Shanghai, , China

Site Status

Shenzhen Children's Hospital

Shenzhen, , China

Site Status

Queen Mary Hospital

Hong Kong, , Hong Kong

Site Status

Panchshil Hospital

Ahmedabad, Gujarat, India

Site Status

National Institute of Mental Health and Neurosciences

Bengaluru, Karnataka, India

Site Status

P.D. Hinduja Hospital

Māhīm, Maharashtra, India

Site Status

Apollo Children's Hospital Chennai

Chennai, Tamil Nadu, India

Site Status

Christian Medical College Hospital Vellore

Vellore, Tamil Nadu, India

Site Status

Nizam's Institute of Medical Sciences (NIMS)

Hyderabad, Telangana, India

Site Status

Apollo Gleneagles Hospital

Kolkata, West Bengal, India

Site Status

Postgraduate Institute of Medical Education and Research

Chandigarh, , India

Site Status

All India Institute of Medical Sciences

New Delhi, , India

Site Status

PTC Clinical Site

Multiple Locations, , Japan

Site Status

Hospital Tunku Azizah Kuala Lumpur

Kuala Lumpur, , Malaysia

Site Status

University Malaya Medical Centre (UMMC)

Pantai, , Malaysia

Site Status

Hospital Angeles Chihuahua

Chihuahua City, , Mexico

Site Status

Instituto Nacional de Pediatría

Mexico City, , Mexico

Site Status

Instituto Nacional de Rehabilitacion

Tlalpan, , Mexico

Site Status

Samodzielny Publiczny Centralny Szpital Kliniczny w Warszawie

Warsaw, , Poland

Site Status

University of Puerto Rico - School of Medicine

San Juan, , Puerto Rico

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

Pusan National University Yangsan Hospital

Yangsan, Gyeongsangnam-do, South Korea

Site Status

Samsung Medical Center

Seoul, , South Korea

Site Status

Seoul National University Hospital

Seoul, , South Korea

Site Status

Kaohsiung Medical University Chung-Ho Memorial Hospital

Kaohsiung City, , Taiwan

Site Status

National Taiwan University Hospital

Taipei, , Taiwan

Site Status

Siriraj Hospital

Bangkok, , Thailand

Site Status

Istanbul University- Instanbul Medical Faculty

Istanbul, , Turkey (Türkiye)

Site Status

Countries

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United States Australia Brazil Bulgaria Canada China Hong Kong India Japan Malaysia Mexico Poland Puerto Rico Russia South Korea Taiwan Thailand Turkey (Türkiye)

Related Links

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Other Identifiers

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PTC124-GD-041-DMD

Identifier Type: -

Identifier Source: org_study_id

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