DMCRN-02-001: Assessing Pediatric Endpoints in DM1

NCT ID: NCT05224778

Last Updated: 2025-06-11

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

Get a concise snapshot of the trial, including recruitment status, study phase, enrollment targets, and key timeline milestones.

Recruitment Status

RECRUITING

Total Enrollment

50 participants

Study Classification

OBSERVATIONAL

Study Start Date

2022-08-24

Study Completion Date

2026-12-31

Brief Summary

Review the sponsor-provided synopsis that highlights what the study is about and why it is being conducted.

The overall goal of the study is to establish valid clinical endpoint assessments for children with congenital myotonic dystrophy type 1 and develop biomarkers for the condition.

Detailed Description

Dive into the extended narrative that explains the scientific background, objectives, and procedures in greater depth.

Myotonic dystrophy type-1 (DM1) is an autosomal dominant disorder caused by a toxic CTG repeat expansion in the 3'UTR of the DMPK gene. DM1 is the most common adult-onset muscular dystrophy, with an overall prevalence of 1:8000. In approximately 10-20% of individuals with DM1, the onset of symptoms occurs at birth, which is known as congenital myotonic dystrophy (CDM).

Previous studies have enrolled a very limited number of children with CDM.

The rationale for this study is to include a larger population of patients with CDM in order to determine developmental milestones, measures of physical and cognitive function and quality of life, and correlate functional outcome measures with potential biomarkers in CDM .

Conditions

See the medical conditions and disease areas that this research is targeting or investigating.

Congenital Myotonic Dystrophy CDM

Study Design

Understand how the trial is structured, including allocation methods, masking strategies, primary purpose, and other design elements.

Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

Review each arm or cohort in the study, along with the interventions and objectives associated with them.

Congenital Myotonic Dystrophy (CDM)

CDM group includes those aged neonate to 3 years, 11 months at enrollment. Individuals must have a diagnosis of CDM, which is defined as children having symptoms of myotonic dystrophy in the newborn period (\<30 days), such as hypotonia, feeding or respiratory difficulty, requiring hospitalization to a ward or to the neonatal intensive care unit for more than 72 hours; and a genetic test confirming an expanded trinucleotide (CTG) repeat in the DMPK gene in the child or mother. An expanded CTG repeat size in the child is considered greater than 200 repeats or E1-E4 classification (E1= 200-500, E2=500-1,000, E3=1,000-1,500, E4\>1,500).

No interventions assigned to this group

Eligibility Criteria

Check the participation requirements, including inclusion and exclusion rules, age limits, and whether healthy volunteers are accepted.

Inclusion Criteria

* Age neonate to 3 years 11 months at enrollment.
* A diagnosis of CDM, which is defined as children having symptoms of myotonic dystrophy in the newborn period (\<30 days), such as hypotonia, feeding or respiratory difficulty, requiring hospitalization to a ward or to the neonatal intensive care unit for more than 72 hours; and a genetic test confirming an expanded trinucleotide (CTG) repeat in the DMPK gene in the child or mother. An expanded CTG repeat size in the child is considered greater than 200 repeats or E1-E4 classification (E1= 200-500, E2=500-1,000, E3=1,000-1,500, E4\>1,500).
* Guardian is willing and able to sign consent and follow study procedures

Exclusion Criteria

* Any other non-DM1 illness that would interfere with the ability or results of the study in the opinion of the site investigator
* Significant trauma within one month
* Internal metal or devices (exclusion for DEXA component)
* History of bleeding disorder or platelet count \<50,000
* History of reaction to local anesthetic
Maximum Eligible Age

59 Months

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

Meet the organizations funding or collaborating on the study and learn about their roles.

Virginia Commonwealth University

OTHER

Sponsor Role lead

Responsible Party

Identify the individual or organization who holds primary responsibility for the study information submitted to regulators.

Responsibility Role SPONSOR

Principal Investigators

Learn about the lead researchers overseeing the trial and their institutional affiliations.

Nicholas E. Johnson, MD

Role: PRINCIPAL_INVESTIGATOR

Virginia Commonwealth University

Locations

Explore where the study is taking place and check the recruitment status at each participating site.

University of California, Los Angeles

Los Angeles, California, United States

Site Status RECRUITING

University of Kansas Medical Center

Fairway, Kansas, United States

Site Status RECRUITING

University of Rochester Medical Center

Rochester, New York, United States

Site Status RECRUITING

Virginia Commonwealth University

Richmond, Virginia, United States

Site Status RECRUITING

Centro Clinico NeMO

Milan, , Italy

Site Status RECRUITING

Countries

Review the countries where the study has at least one active or historical site.

United States Italy

Central Contacts

Reach out to these primary contacts for questions about participation or study logistics.

Ruby Langeslay

Role: CONTACT

804-828-8481

Jennifer Raymond

Role: CONTACT

804-828-6318

Facility Contacts

Find local site contact details for specific facilities participating in the trial.

Merve Kaleli

Role: primary

Dennis Fernando

Role: backup

310-825-3264

Cassidy Nelson

Role: primary

Michaela Walker

Role: backup

Jim Hilbert

Role: primary

Jeanne Dekdebrun

Role: backup

Shantel Kyles

Role: primary

Jodie Howell

Role: backup

Michela Nani

Role: primary

Luca Mauro

Role: backup

Other Identifiers

Review additional registry numbers or institutional identifiers associated with this trial.

HM20023386

Identifier Type: -

Identifier Source: org_study_id

More Related Trials

Additional clinical trials that may be relevant based on similarity analysis.

Phase I/II Study of SRP-4053 in DMD Patients
NCT02310906 COMPLETED PHASE1/PHASE2
Study of Eteplirsen in DMD Patients
NCT02255552 COMPLETED PHASE3
NS-050/NCNP-03 in Boys With DMD (Meteor50)
NCT06053814 RECRUITING PHASE1/PHASE2