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Trial details imported from ClinicalTrials.gov

For full trial details, please see the original record at https://clinicaltrials.gov/study/NCT05004129




Registration number
NCT05004129
Ethics application status
Date submitted
5/08/2021
Date registered
13/08/2021
Date last updated
8/11/2023

Titles & IDs
Public title
Safety and Efficacy of Tideglusib in Congenital or Childhood Onset Myotonic Dystrophy
Scientific title
An Open-Label Study to Evaluate the Long-Term Safety and Efficacy of Tideglusib for the Treatment of Congenital or Childhood Onset DM1 (REACH CDM X)
Secondary ID [1] 0 0
AMO-02-MD-2-004
Universal Trial Number (UTN)
Trial acronym
REACH CDM X
Linked study record

Health condition
Health condition(s) or problem(s) studied:
Congenital Myotonic Dystrophy 0 0
Condition category
Condition code
Musculoskeletal 0 0 0 0
Other muscular and skeletal disorders

Intervention/exposure
Study type
Interventional
Description of intervention(s) / exposure
Treatment: Drugs - Tideglusib

Experimental: Tideglusib - Weight adjusted tideglusib, orally, once daily


Treatment: Drugs: Tideglusib
All subjects will receive weight-adjusted 1000 mg tideglusib following a titration period of 2 weeks at a weight-adjusted dose of 400 mg and 2 weeks at a weight-adjusted dose of 600 mg tideglusib.

Intervention code [1] 0 0
Treatment: Drugs
Comparator / control treatment
Control group

Outcomes
Primary outcome [1] 0 0
Safety (Adverse Events)
Timepoint [1] 0 0
52 Weeks
Primary outcome [2] 0 0
Safety (Adverse Events) - With Optional Expanded Access
Timepoint [2] 0 0
Week 60 and every 8 weeks thereafter up until discontinuation or study closure, assessed up to Week 132
Primary outcome [3] 0 0
Clinician-Completed Congenital DM1 Rating Scale (CDM1-RS)
Timepoint [3] 0 0
52 Weeks
Secondary outcome [1] 0 0
Clinician-Completed Congenital DM1 Rating Scale (CDM1-RS) - With Optional Expanded Access
Timepoint [1] 0 0
Week 68 and every 16 weeks thereafter up until discontinuation or study closure, assessed up to Week 132
Secondary outcome [2] 0 0
Clinical Global Impressions Improvement Scale (CGI-I)
Timepoint [2] 0 0
54 Weeks
Secondary outcome [3] 0 0
Clinical Global Impressions Improvement Scale (CGI-I) - With Optional Expanded Access
Timepoint [3] 0 0
Week 68 and every 16 weeks thereafter up until discontinuation or study closure, assessed up to Week 132
Secondary outcome [4] 0 0
Top 3 Caregiver Concerns Visual Analogue Scale (VAS) score
Timepoint [4] 0 0
54 weeks
Secondary outcome [5] 0 0
Top 3 Caregiver Concerns Visual Analogue Scale (VAS) score - With Optional Expanded Access
Timepoint [5] 0 0
Week 68 and every 16 weeks thereafter up until discontinuation or study closure, assessed up to Week 132
Secondary outcome [6] 0 0
Caregiver Completed Congenital DM1 Rating Scale (CC-CDM1-RS)
Timepoint [6] 0 0
52 weeks
Secondary outcome [7] 0 0
Caregiver Completed Congenital DM1 Rating Scale (CC-CDM1-RS) - With Optional Expanded Access
Timepoint [7] 0 0
Week 68 and every 16 weeks thereafter up until discontinuation or study closure, assessed up to Week 132
Secondary outcome [8] 0 0
Clinical Global Impressions Severity Scale (CGI-S)
Timepoint [8] 0 0
54 weeks
Secondary outcome [9] 0 0
Clinical Global Impressions Severity Scale (CGI-S) - With Optional Expanded Access
Timepoint [9] 0 0
Week 68 and every 16 weeks thereafter up until discontinuation or study closure, assessed up to Week 132
Secondary outcome [10] 0 0
Autism Behavior Inventory- Clinician (ABI-C)
Timepoint [10] 0 0
52 Weeks
Secondary outcome [11] 0 0
Socialization, Communication, Daily Living, and Adaptive Behavior Composite standard scores of the Vineland Adaptive Behavior Scale - Survey Interview
Timepoint [11] 0 0
52 Weeks
Secondary outcome [12] 0 0
10-meter walk-run test
Timepoint [12] 0 0
52 Weeks
Secondary outcome [13] 0 0
Plasma Troponin T levels
Timepoint [13] 0 0
52 Weeks
Secondary outcome [14] 0 0
Plasma Troponin T levels - With Optional Expanded Access
Timepoint [14] 0 0
Week 68 and every 16 weeks thereafter up until discontinuation or study closure, assessed up to Week 132

Eligibility
Key inclusion criteria
Subjects who do not enter this study directly from completing the AMO-02-MD-2-003 study (i.e. subjects who did not complete AMO-02-MD-2-003, subjects who completed AMO-02-MD-2-003 but did not directly rollover or subjects who are re-entering AMO-02-MD-2-004), will not be considered eligible for the study without meeting all of the criteria below:

1. Subjects under study must be individuals with a diagnosis of Congenital or Childhood Onset DM1.
2. Diagnosis must be genetically confirmed
3. Subjects must be male or female aged =6 years to =45 years at Screening
4. Subjects must have a Clinical Global Impression - Severity (CGI-S) score of 3 or greater at Screening (V-1)
5. Written, voluntary informed consent must be obtained before any study related procedures are conducted. Where a parent or legally authorized representative (LAR) provides consent, there must also be assent from the subject (as required by local regulations)
6. Subject's caregiver must be willing and able to support participation for duration of study
7. Subject must be willing and able to comply with the required food intake restrictions as outlined per protocol

Subjects entering directly from completing the antecedent AMO-02-MD-2-003 study will not be considered eligible for the study without meeting all of the criteria below:

1. Subjects who have completed the antecedent AMO-02-MD-2-003 study through V11
2. Written, voluntary informed consent must be obtained before any study related procedures are conducted. Where a parent or LAR provides consent, there must also be assent from the subject (as required by local regulations)
3. Subject's caregiver must be willing and able to support participation for duration of study
4. Subject must be willing and able to comply with the required food intake restrictions as outlined per protocol

Key
Minimum age
6 Years
Maximum age
45 Years
Sex
Both males and females
Can healthy volunteers participate?
No
Key exclusion criteria
1. Body mass index (BMI) less than 13.5 kg/m² or greater than 40 kg/m²
2. New or change in medications/therapies within 4 weeks prior to Eligibility/Baseline Visit
3. Use within 4 weeks prior to Eligibility/Baseline Visit of strong CYP3A4 inhibitors (eg.clarithromycin, telithromycin, ketoconazole, itraconazole, posaconazole, nefazodone, idinavir and ritonavir)
4. Concurrent use of drugs metabolized by CYP3A4 with a narrow therapeutic window (e.g. warfarin and digitoxin)
5. Current enrollment in a clinical trial of an investigational drug or enrollment in a clinical trial of an investigational drug in the last 6 months other than the AMO-02- MD-2-003 study
6. Existing or historical medical conditions or complications (eg. neurological, cardiovascular, renal, hepatic, gastrointestinal, endocrine or respiratory disease) that may impact the interpretability of the study results
7. Hypersensitivity to tideglusib or any components of its formulation including allergy to strawberry

Study design
Purpose of the study
Treatment
Allocation to intervention
NA
Procedure for enrolling a subject and allocating the treatment (allocation concealment procedures)
Methods used to generate the sequence in which subjects will be randomised (sequence generation)
Masking / blinding
Open (masking not used)
Who is / are masked / blinded?



Intervention assignment
Single group
Other design features
Phase
Phase 2
Type of endpoint/s
Statistical methods / analysis

Recruitment
Recruitment status
Recruiting
Data analysis
Reason for early stopping/withdrawal
Other reasons
Date of first participant enrolment
Anticipated
Actual
Date of last participant enrolment
Anticipated
Actual
Date of last data collection
Anticipated
Actual
Sample size
Target
Accrual to date
Final
Recruitment in Australia
Recruitment state(s)
NSW
Recruitment hospital [1] 0 0
The Bright Alliance - Randwick
Recruitment postcode(s) [1] 0 0
2031 - Randwick
Recruitment outside Australia
Country [1] 0 0
United States of America
State/province [1] 0 0
Arkansas
Country [2] 0 0
United States of America
State/province [2] 0 0
California
Country [3] 0 0
United States of America
State/province [3] 0 0
Illinois
Country [4] 0 0
United States of America
State/province [4] 0 0
Iowa
Country [5] 0 0
United States of America
State/province [5] 0 0
New York
Country [6] 0 0
United States of America
State/province [6] 0 0
Pennsylvania
Country [7] 0 0
United States of America
State/province [7] 0 0
Utah
Country [8] 0 0
United States of America
State/province [8] 0 0
Virginia
Country [9] 0 0
Canada
State/province [9] 0 0
Ontario
Country [10] 0 0
New Zealand
State/province [10] 0 0
Auckland

Funding & Sponsors
Primary sponsor type
Commercial sector/industry
Name
AMO Pharma Limited
Address
Country

Ethics approval
Ethics application status

Summary
Brief summary
Trial website
Trial related presentations / publications
Public notes

Contacts
Principal investigator
Name 0 0
Joseph P Horrigan, MD
Address 0 0
AMO Pharma
Country 0 0
Phone 0 0
Fax 0 0
Email 0 0
Contact person for public queries
Name 0 0
Address 0 0
Country 0 0
Phone 0 0
Fax 0 0
Email 0 0
Contact person for scientific queries

No information has been provided regarding IPD availability


What supporting documents are/will be available?

No Supporting Document Provided



Results publications and other study-related documents

No documents have been uploaded by study researchers.