This is a controlled, open-label, single-ascending dose study to evaluate the safety and tolerability of SGT-001 in adolescents and children with Duchenne muscular dystrophy (DMD). Participants will receive a single intravenous (IV) infusion of SGT-001 and will be followed for approximately 5 years. The protocol was amended to drop the control arm after 4 participants were dosed.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
12
AAV9 vector containing muscle-specific promoter and microdystrophin construct
David Geffen School of Medicine at UCLA
Los Angeles, California, United States
University of Florida
Gainesville, Florida, United States
Number of Participants with Treatment Emergent Adverse Events (TEAEs)
Time frame: Up to 5 years
Number of Participants with Clinically Significant Abnormalities in Laboratory Parameters
Time frame: Up to 5 years
Number of Participants with Clinically Significant Abnormalities in Vital Signs
Time frame: Up to 5 years
Number of Participants with Clinically Significant Abnormalities in Physical Examinations
Time frame: Up to 5 years
Number of Participants with Clinically Significant Abnormalities in Electrocardiogram (ECG)
Time frame: Up to 5 years
Change from Baseline in Microdystrophin Protein Levels in Muscle Biopsies Using Western Blot (WB)
Time frame: Baseline, 12 months
Change from Baseline in Microdystrophin Protein Levels in Muscle Biopsies Using Immunofluorescence (IF)
Time frame: Baseline, 12 months
Change from Baseline in North Star Ambulatory Assessment (NSAA) score in Ambulatory Participants
Time frame: Baseline, 12 months
Change from Baseline in 6-minute walk test (6MWT) Distance in Ambulatory Participants
Time frame: Baseline, 12 months
Change from Baseline in Total Upper Limb Function, as Measured by the Total Performance of the Upper Limb (PUL) Functional Scale Score
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Time frame: Baseline, 12 months
Change from Baseline in Respiratory Function, as Measured by Forced Vital Capacity (FVC) % Predicted, Forced Expiratory Volume in 1 second (FEV1) % Predicted, and Peak Expiratory Flow (PEF) % Predicted
Time frame: Baseline, 12 months
Change from Baseline in Ejection Fraction, As Measured by Echocardiography
Time frame: Baseline,12 months
Change from Baseline in Left Ventricular End Systolic Volume, As Measured by Echocardiography
Time frame: Baseline,12 months
Change from Baseline in Myocardial Peak Circumferential Strain (Ecc), As Measured by Echocardiography
Time frame: Baseline,12 months
Change from Baseline in Quality of Life as Measured by the Paediatric Quality of Life Inventory (PedsQL) Duchenne muscular dystrophy (DMD) module and self-reported outcome measures as measured by the PODCI DMD module
Time frame: Baseline, 12 months