RecruitingNCT05224778

DMCRN-02-001: Assessing Pediatric Endpoints in DM1

Assessing Pediatric Endpoints in DM1 (ASPIRE-DM1)


Sponsor

Virginia Commonwealth University

Enrollment

50 participants

Start Date

Aug 24, 2022

Study Type

OBSERVATIONAL

Conditions

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.


Eligibility

Max Age: 59 Months

Inclusion Criteria3

  • 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 Criteria5

  • 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

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Locations(5)

University of California, Los Angeles

Los Angeles, California, United States

University of Kansas Medical Center

Fairway, Kansas, United States

University of Rochester Medical Center

Rochester, New York, United States

Virginia Commonwealth University

Richmond, Virginia, United States

Centro Clinico NeMO

Milan, Italy

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NCT05224778


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