An Extension Study of Ataluren (PTC124) in Participants With Nonsense Mutation Dystrophinopathy

NCT ID: NCT02090959

Last Updated: 2020-08-11

Study Results

Results available

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Basic Information

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Recruitment Status

TERMINATED

Clinical Phase

PHASE3

Total Enrollment

219 participants

Study Classification

INTERVENTIONAL

Study Start Date

2014-03-20

Study Completion Date

2018-06-12

Brief Summary

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The primary objective of this study is to obtain long term safety data of ataluren in male participants with nonsense mutation dystrophinopathy (who participated and completed a previous Phase 3 study of ataluren \[PTC124-GD-020-DMD {NCT01826487}\]) to augment the overall safety database. Screening and baseline procedures are structured to avoid a gap in treatment between the double-blind study (PTC124-GD-020-DMD) and this extension study.

This study may be further extended by amendment until either ataluren becomes commercially available or the clinical development of ataluren in duchenne muscular dystrophy (DMD) is discontinued.

Detailed Description

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Conditions

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

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Ataluren

Participants will receive ataluren suspension orally 3 times a day (TID), 10 milligrams/kilogram (mg/kg) at morning, 10 mg/kg at midday, and 20 mg/kg at evening (total daily dose 40 mg/kg) for up to 144 weeks.

Group Type EXPERIMENTAL

Ataluren

Intervention Type DRUG

Ataluren will be administered per the dose and schedule specified in the arm.

Interventions

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Ataluren

Ataluren will be administered per the dose and schedule specified in the arm.

Intervention Type DRUG

Other Intervention Names

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PTC124

Eligibility Criteria

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Inclusion Criteria

* Completion of study treatment in the previous Phase 3, double-blind study (PTC124-GD-020-DMD).
* Evidence of signed and dated informed consent/assent document(s) indicating that the participant (and/or his parent/legal guardian) has been informed of all pertinent aspects of the trial. Note: If the study candidate is considered a child under local regulation, a parent or legal guardian must provide written consent prior to initiation of study screening procedures and the study candidate may be required to provide written assent. The rules of the responsible Institutional Review Board/Independent Ethic Committee (IRB/IEC) regarding whether 1 or both parents must provide consent and the appropriate ages for obtaining consent and assent from the participant should be followed.
* In participants who are sexually active, willingness to abstain from sexual intercourse or employ a barrier or medical method of contraception during the period of study drug administration and 6-week follow-up period.
* Willingness and ability to comply with scheduled visits, ataluren administration plan, study procedures, laboratory tests, and study restrictions.

Exclusion Criteria

* Known hypersensitivity to any of the ingredients or excipients of the study drug (Litesse® UltraTM \[refined polydextrose\], polyethylene glycol 3350, Lutrol® micro F127 \[poloxamer 407\], mannitol 25C, crospovidone XL10, hydroxyethyl cellulose, vanilla, Cab-O-Sil® M5P \[colloidal silica\], and magnesium stearate).
* Ongoing participation in any other therapeutic clinical trial.
* Prior or ongoing medical condition (for example, concomitant illness, psychiatric condition, behavioral disorder, alcoholism, drug abuse), medical history, physical findings, electrocardiogram (ECG) findings, or laboratory abnormality that, in the investigator's opinion, could adversely affect the safety of the participant, makes it unlikely that the course of treatment or follow-up would be completed, or could impair the assessment of study results.
Minimum Eligible Age

7 Years

Maximum Eligible Age

15 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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Francesco Bibbiani, M.D.

Role: STUDY_DIRECTOR

PTC Therapeutics

Locations

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University of California, Los Angeles

Los Angeles, California, United States

Site Status

UC Davis Medical Center

Sacramento, California, United States

Site Status

Stanford University Medical Center

Stanford, California, United States

Site Status

Children's Hospital Colorado - Center for Cancer and Blood Disorders

Aurora, Colorado, United States

Site Status

Child Neurology Center of Northwest Florida

Gulf Breeze, Florida, United States

Site Status

Rush University Medical Center

Chicago, Illinois, United States

Site Status

University of Iowa

Iowa City, Iowa, United States

Site Status

University of Kansas Medical Center

Kansas City, Kansas, United States

Site Status

Children's Hospital Boston

Boston, Massachusetts, United States

Site Status

University of Minnesota

Minneapolis, Minnesota, United States

Site Status

Washington University School of Medicine, Division of Endocrinology, Metabolism and Lipid Research

St Louis, Missouri, United States

Site Status

Columbia University College of Physicians & Surgeons

New York, New York, United States

Site Status

Children's Hospital Cincinnati

Cincinnati, Ohio, United States

Site Status

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status

Oregon Health & Science University

Portland, Oregon, United States

Site Status

The Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

Children's Hospital of Pittsburgh

Pittsburgh, Pennsylvania, United States

Site Status

Childrens Medical Center Dallas, Texas

Dallas, Texas, United States

Site Status

Texas Children's Hospital

Houston, Texas, United States

Site Status

University of Utah

Salt Lake City, Utah, United States

Site Status

Seattle Children's Hospital - Childhood Cancer and Blood Disorders

Seattle, Washington, United States

Site Status

The Children's Hospital at Westmead

Westmead, New South Wales, Australia

Site Status

The Royal Children's Hospital

Parkville, Victoria, Australia

Site Status

UZ Leuven

Leuven, , Belgium

Site Status

Universidade Federal do Rio de Janeiro - Instituto de Puericultura e Pediatria Martagao Gesteira

Rio de Janeiro, , Brazil

Site Status

Sao Paulo University -HC/FMUSP

São Paulo, , Brazil

Site Status

Aleksandrovska Hospital

Sofia, , Bulgaria

Site Status

Alberta Children's Hospital

Calgary, Alberta, Canada

Site Status

British Columbia Children's Hospital

Vancouver, British Columbia, Canada

Site Status

Children's Hospital of Western Ontario

London, Ontario, Canada

Site Status

Hospital Luis Calvo Mackenna

Santiago, Santiago Metropolitan, Chile

Site Status

Hospital Clinico Universidad Catolica

Santiago, , Chile

Site Status

University Hospital Brno

Brno, , Czechia

Site Status

Motol University Hospital

Prague, , Czechia

Site Status

Hospital de la Timone

Marseille, , France

Site Status

CHU de Nantes

Nantes, , France

Site Status

Hopital Necker - Enfants Malades

Paris, , France

Site Status

Groupe Hospitalier Pitie-Salpetriere

Paris, , France

Site Status

Universitaetsklinikum Essen

Essen, , Germany

Site Status

University Medical Center Freiburg

Freiburg im Breisgau, , Germany

Site Status

Universitat Munchen - von Haunersche Kinder Clinic

München, , Germany

Site Status

Hadassah University Hospital

Jerusalem, , Israel

Site Status

Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico di Milano

Milan, , Italy

Site Status

Bambino Gesu Hospital

Rome, , Italy

Site Status

U.O. Complessa di Neuropsichiatria Infantile

Rome, , Italy

Site Status

Policlinico Universitario G. Martino

Sicily, , Italy

Site Status

Medical University of Warsaw

Warsaw, , Poland

Site Status

Seoul National University Hospital

Seoul, , South Korea

Site Status

Hospital Sant Joan de Deu

Barcelona, , Spain

Site Status

Hospital Universitari i Politecnic la Fe

Valencia, , Spain

Site Status

Queen Silvia Children's Hospital

Gothenburg, , Sweden

Site Status

Astrid Lindgren Childrens Hospital

Stockholm, , Sweden

Site Status

CHUV Lausanne

Lausanne, , Switzerland

Site Status

Hacettepe Childrens Hospital

Ankara, , Turkey (Türkiye)

Site Status

Erciyes University Faculty of Medicine

Talas/Kayseri, , Turkey (Türkiye)

Site Status

University College London Institute of Child Health

London, , United Kingdom

Site Status

Royal Manchester Children's Hospital

Manchester, , United Kingdom

Site Status

The Newcastle upon Tyne Hospitals, NHS Foundation Trust

Newcastle upon Tyne, , United Kingdom

Site Status

Countries

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United States Australia Belgium Brazil Bulgaria Canada Chile Czechia France Germany Israel Italy Poland South Korea Spain Sweden Switzerland Turkey (Türkiye) United Kingdom

References

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Thangarajh M, Elfring GL, Trifillis P, McIntosh J, Peltz SW; Ataluren Phase 2b Study Group. The relationship between deficit in digit span and genotype in nonsense mutation Duchenne muscular dystrophy. Neurology. 2018 Sep 25;91(13):e1215-e1219. doi: 10.1212/WNL.0000000000006245. Epub 2018 Aug 22.

Reference Type DERIVED
PMID: 30135256 (View on PubMed)

Provided Documents

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Document Type: Study Protocol

View Document

Document Type: Statistical Analysis Plan

View Document

Related Links

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Other Identifiers

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PTC124-GD-020e-DMD

Identifier Type: -

Identifier Source: org_study_id

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