Status:

RECRUITING

DMCRN-02-001: Assessing Pediatric Endpoints in DM1

Lead Sponsor:

Virginia Commonwealth University

Conditions:

Congenital Myotonic Dystrophy

CDM

Eligibility:

All Genders

Up to 59 years

Brief Summary

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

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 o...

Eligibility Criteria

Inclusion

  • 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

  • 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

Key Trial Info

Start Date :

August 24 2022

Trial Type :

OBSERVATIONAL

Allocation :

ESTIMATED

End Date :

December 1 2026

Estimated Enrollment :

50 Patients enrolled

Trial Details

Trial ID

NCT05224778

Start Date

August 24 2022

End Date

December 1 2026

Last Update

June 11 2025

Active Locations (5)

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Page 1 of 2 (5 locations)

1

University of California, Los Angeles

Los Angeles, California, United States, 90095

2

University of Kansas Medical Center

Fairway, Kansas, United States, 66205

3

University of Rochester Medical Center

Rochester, New York, United States, 14642

4

Virginia Commonwealth University

Richmond, Virginia, United States, 23298