Observation Study in Patients Age 0-5 Years With LAMA2-related Congenital Muscular Dystrophy

NCT ID: NCT06503367

Last Updated: 2025-08-12

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

RECRUITING

Total Enrollment

44 participants

Study Classification

OBSERVATIONAL

Study Start Date

2025-05-12

Study Completion Date

2028-09-30

Brief Summary

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The goal of this observational study is to understand how young children with LAMA2-related dystrophy move and change over time. We will also learn about how this condition impacts other body systems.

Participants will undergo:

* Neuromuscular assessments
* Blood collections
* Swallowing and breathing assessments
* Questionnaires

Detailed Description

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READY CMD LAMA2 is a Pre-Phase 1, single-arm, multicenter, prospective natural history clinical study to evaluate the natural history and potential early outcome measures in subjects with laminin α2-related dystrophy (LAMA2-RD) aged 0-5 years. The study seeks to enroll 44 subjects across 14 sites. NCH will enroll 10 subjects.

Subjects will complete in-person visits at Baseline, Month 6, Month 12, Month 18, and Month 24. Within two weeks of the Baseline, Month 12, and Month 24 visits, a remote visit will be performed to repeat and compare remote outcomes with in-person outcomes. Remote visits only will also be performed at Month 3 and Month 9. Adverse events will be captured every three months, beginning at baseline. Phone calls will be made by the coordinator and/or other delegated study staff at months 3, 9, 15, and 21.

The total study duration for each subject is up to 24 months.

Physical assessments to be performed will depend on the subject's age and may include the following:

* Assessment of motor milestones (maintained, lost or acquired)
* Neuromuscular Gross Motor Outcome (GRO)
* Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND)
* Motor Function Measure (MFM-20)
* Bayley-4
* Respiratory assessments: capnography and collection of data on use of non- invasive ventilation and other respiratory support
* Physical exam
* Swallow/oromotor assessment: To be performed by physician, occupational therapist, speech language pathologist, or other licensed professional at the site.
* Muscle ultrasound may be performed at some sites.

Other assessments:

* Growth parameters \[head circumference, chest circumference, length, weight, body mass index (BMI)\]
* 12-lead electrocardiogram
* Mode of feeding
* Peds Quality of Life (PedsQL) Neuromuscular Module
* Pediatric Evaluation of Disability Inventory (PEDI)
* Clinical Global Impression of Change (CGIC), physician and parent/caregiver assessed.
* Blood samples will be collected from subjects and stored for biomarker and other research purposes. Blood samples will be collected at each in-person visit.
* Data from electronic medical records of evaluations performed as standard of care visits will be collected.

Medical history, concomitant medications, and AEs will be collected and assessed for a potential relationship to participation in this study.

The total duration of the study for each subject is up to approximately 24 months. A subject will be considered to have competed the study if he/she has completed all assessments up to and including Month 24 assessments. The end of the study is defined as the date of completion of the last scheduled assessment shown in the Schedule of Events for the last subject in the study.

Subjects who prematurely discontinue study participation will be encouraged to complete an End-of- Study Visit to include assessment of motor milestones and recording of AEs.

Conditions

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LAMA2-MD \(Merosin Deficient Congenital Muscular Dystrophy, MDC1A\)

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Eligibility Criteria

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Inclusion Criteria

* Signed informed consent by the subject, parent(s) or legally authorized representative (LAR) and/or assent by the subject (when applicable).
* Subject must be aged birth to less than 5.0 years of age at time of consent.
* A confirmed diagnosis of LAMA2-RD confirmed via:

a: Two pathogenic variants in the LAMA2 gene (via a CLIA-approved laboratory) or: b. muscle biopsy with absence of merosin (laminin-211) and at least one pathogenic variant in the LAMA2 gene
* Absence of another confirmed genetic disease.
* Willingness to maintain current exercise and/or physical therapy regimen for the duration of the clinical study.
* Willingness to comply with the study protocol, including but not limited to, all study procedures and visits.

Exclusion Criteria

* Acute medical illness or hospitalization within 30 days prior to informed consent.
* Participation in a previous trial of any investigational agent for LAMA2-RD within 1 month prior to informed consent, or use of any other investigational therapy (including off-label use of Losartan) within 30 days prior to informed consent, or participation in other clinical studies, within 30 days (or 3 half-lives, whichever is longer) prior to informed consent, which in the opinion of the PI, may potentially confound results from this study.
* Other significant medical condition, which in the opinion of the site Principal Investigator may confound interpretation of the clinical course of LAMA2- RD.
Maximum Eligible Age

5 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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National Institute of Neurological Disorders and Stroke (NINDS)

NIH

Sponsor Role collaborator

Ann & Robert H Lurie Children's Hospital of Chicago

OTHER

Sponsor Role collaborator

Children's Hospital Medical Center, Cincinnati

OTHER

Sponsor Role collaborator

University of Iowa

OTHER

Sponsor Role collaborator

Washington University School of Medicine

OTHER

Sponsor Role collaborator

Stanford University

OTHER

Sponsor Role collaborator

University of Utah

OTHER

Sponsor Role collaborator

University of Rochester

OTHER

Sponsor Role collaborator

University of Pittsburgh

OTHER

Sponsor Role collaborator

University of Texas, Southwestern Medical Center at Dallas

OTHER

Sponsor Role collaborator

University of Minnesota

OTHER

Sponsor Role collaborator

University of California, Los Angeles

OTHER

Sponsor Role collaborator

Boston Children's Hospital

OTHER

Sponsor Role collaborator

Nationwide Children's Hospital

OTHER

Sponsor Role lead

Responsible Party

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Anne M. Connolly

Principle Investigator

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Anne M Connolly, MD

Role: PRINCIPAL_INVESTIGATOR

Nationwide Childrens Hospital

Locations

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University of California, Los Angeles

Los Angeles, California, United States

Site Status RECRUITING

Stanford University

Stanford, California, United States

Site Status RECRUITING

Ann & Robert H. Lurie Children's Hospital of Chicago

Chicago, Illinois, United States

Site Status RECRUITING

University of Iowa Stead Family Children's Hospital

Iowa City, Iowa, United States

Site Status RECRUITING

National Institute of Neurological Disorders and Stroke, NIH

Bethesda, Maryland, United States

Site Status NOT_YET_RECRUITING

Boston Children's Hospital

Boston, Massachusetts, United States

Site Status RECRUITING

University of Minnesota

Saint Paul, Minnesota, United States

Site Status RECRUITING

Washington University in St. Louis

St Louis, Missouri, United States

Site Status RECRUITING

University of Rochester

Rochester, New York, United States

Site Status RECRUITING

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status RECRUITING

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status RECRUITING

Children's Hospital of Pittsburgh

Pittsburgh, Pennsylvania, United States

Site Status NOT_YET_RECRUITING

University of Texas Southwestern

Dallas, Texas, United States

Site Status RECRUITING

University of Utah

Salt Lake City, Utah, United States

Site Status NOT_YET_RECRUITING

Countries

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United States

Central Contacts

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Kevin Warf

Role: CONTACT

(614) 355-2765

Facility Contacts

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Indumathi Mohanchandra

Role: primary

(310) 825-3264

Lin Karman

Role: primary

(650) 384-9417

Raven Hill

Role: primary

(312) 227-2937

Laura Knosp

Role: primary

(319) 384-6277

Alexa Yarish

Role: primary

(301) 402-2273

Peter Riley

Role: primary

(617) 919-7384

Ellen Poppy

Role: primary

(612) 625-2850

Pallavi Anand

Role: primary

(314) 362-1566

Kim Hart

Role: primary

(585) 275-3767

Jessica Getz

Role: primary

(513) 803-9036

Kevin Warf

Role: primary

(614) 355-2765

Heather Dicostanzo

Role: primary

(412) 692-7660

Shahera Ranjha

Role: primary

(214) 456-5959

Jessi Beyer

Role: primary

(801) 587-4865

Other Identifiers

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1U01NS124961-01A1

Identifier Type: NIH

Identifier Source: secondary_id

View Link

Ready CMD LAMA2

Identifier Type: -

Identifier Source: org_study_id

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