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Phase 3 study to evaluate the safety and efficacy of PF-06939926 for the treatment of Duchenne muscular dystrophy.

Phase 1
Conditions
DUCHENNE MUSCULAR DYSTROPHY (DMD)
MedDRA version: 20.0Level: PTClassification code 10013801Term: Duchenne muscular dystrophySystem Organ Class: 10010331 - Congenital, familial and genetic disorders
Therapeutic area: Diseases [C] - Musculoskeletal Diseases [C05]
Registration Number
EUCTR2019-002921-31-IT
Lead Sponsor
PFIZER INC
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
Authorised-recruitment may be ongoing or finished
Sex
Male
Target Recruitment
99
Inclusion Criteria

- Male participants who are =4 and <8 years of age at Screening (Visit 1).
- Confirmed diagnosis of DMD by prior genetic testing demonstrating the presence of a mutation in the dystrophin gene consistent with DMD at Screening (Visit 1). If the Investigator determines that the results are inconclusive, a repeat genetic testing will be allowed through the central laboratory at Screening (Visit 1).
- Receipt of a stable daily dose of glucocorticoids (=0.5 mg/kg/day prednisone, prednisolone, or =0.75 mg/kg/day deflazacort) for at least 3 months prior to Screening (Visit 1) and during the period between
Screening (Visit 1) and Day 1 (Visit 3). In order to comply with protocol procedures, there should also be a reasonable expectation that this daily dose of glucocorticoids will remain stable for the first 2 years of the study. A stable dose is defined as one in which any change is =0.2 mg/kg
- A NSAA total score >16 and <30 at Screening (Visit 1).
- Ambulatory, defined as being able to walk 10 meters unassisted, at Screening (Visit 1).
- Participants/legally acceptable representatives who are willing and able to comply with all scheduled visits, treatment plan, laboratory tests, lifestyle considerations, and other study procedures including,
potentially, open and/or needle muscle biopsies under general anesthesia.
- Participants/legally acceptable representatives who are capable of giving assent/signed informed consent as described in Appendix 1, which includes compliance with the requirements and restrictions listed in the assent/informed consent document (ICD) and in this protocol.
- Participants/legally acceptable representatives who are willing to protect the integrity of the study data by not actively seeking sensitive clinical data (eg, CK, ALT, AST, NAb to AAV9) through independent
laboratory tests and by not sharing trial experiences with other participants or publicly (eg, through social media).
Are the trial subjects under 18? yes
Number of subjects for this age range:
F.1.2 Adults (18-64 years) no
F.1.2.1 Number of subjects for this age range
F.1.3 Elderly (>=65 years) no
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

- Prior treatment with gene therapy, defined as any therapy introducing exogenous DNA or intended to permanently alter the endogenous DNA. Gene therapy (other than IP) will be prohibited for the duration of the study.
- Exposure within 6 months prior to Screening (Visit 1) to any treatment designed to increase dystrophin expression (including, but not limited to exon-skipping and nonsense read-through). These treatments will also be prohibited during the period between Screening (Visit 1) and Day 1 (Visit 3) and for the first 2 years of the study.
- Previous administration with an investigational drug within 30 days (or as determined by the local requirement) or 5 half-lives (whichever is longer) at Screening (Visit 1). These treatments will also be prohibited during the period between Screening (Visit 1) and Day 1 (Visit 3) and for
the first 2 years of the study.
- Known cognitive impairment or behavioral issues that would impede the ability to follow instructions, in the judgment of the Investigator, at Screening (Visit 1).
- Any nonhealed injury at Screening (Visit 1) which, in the opinion of the Investigator, may impact functional testing; additionally, lower limb fractures must have been healed for at least 3 months prior to Screening (Visit 1).
- Positive test for NAb to AAV9, based on the threshold determined by the Central Laboratory, from a sample taken at Screening (Visit 1).
- Receipt of a live attenuated vaccination within 90 days prior to Screening (Visit 1). Receipt of a live attenuated vaccination will also be prohibited during the period between Screening (Visit 1) and Day 1
(Visit 3), for 90 days prior to Year 2 IP administration, and for the first 2 months after each IP administration.
-Receipt of an influenza vaccination, systemic antiviral and/or interferon therapy within 30 days prior to Screening (Visit 1). Receipt of an influenza vaccination, systemic antiviral and/or interferon therapy will
also be prohibited during the period between Screening (Visit 1) and Day 1 (Visit 3), for 30 days prior to Year 2 IP administration, and for the first 2 months after each IP administration.
-Receipt of any systemic immunosuppressant agents other than glucocorticoids within 30 days prior to Screening (Visit 1). Receipt of any systemic immunosuppressant agents other than glucocorticoids will also be prohibited during the period between Screening (Visit 1) and Day 1 (Visit 3) and during the first 2 years of the study, unless administered in response to immunologic reaction.
-Abnormality in hematology or chemistry profiles at Screening (Visit 1).
A single repeat for value(s) outside allowable limits is permitted to reassess eligibility:
a. Absolute neutrophil count <1000 cells/mm3;
b. Cystatin C >1.0 mg/L;
c. Positive hepatitis A virus (anti-HAV) immunoglobulin M, hepatitis B surface antigen (HBsAg), and/or hepatitis C antibody (HCVAb);
d. Markers of hepatic inflammation or overt or occult cirrhosis as evidenced by one or more of the following:
1. Prothrombin time (PT) > upper limit of normal (ULN); prolonged international normalized ratio (INR) >ULN;
2. GLDH >2 x ULN;
3. Total bilirubin >1.5 x ULN(unless the participant has a history of Gilbert disease) and direct bilirubin >0.5 mg/dL;
4. Gamma-glutamyl transferase (GGT) >1.5 x ULN.
For full list of exclusion criteria please refer to study protocol.

Study & Design

Study Type
Interventional clinical trial of medicinal product
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Main Objective: To demonstrate superior efficacy of treatment with PF-06939926 as compared to placebo based on change from Baseline in the North Star Ambulatory Assessment (NSAA).;Secondary Objective: - To quantify the mini-dystrophin expression level in the muscle of participants treated with PF-06939926.<br>- To characterize the distribution of mini-dystrophin expression in the muscle of participants treated with PF-06939926.<br>- To characterize the change in serum creatine kinase (CK) concentration in participants treated with PF-06939926 as compared to placebo.;Primary end point(s): Change from Baseline at Week 52 in the NSAA total score.;Timepoint(s) of evaluation of this end point: Week 52
Secondary Outcome Measures
NameTimeMethod
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