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Phase 2 N=69 Randomized Quadruple-blind Supportive Care

Safety and Efficacy Study of Recombinant Human Insulin-Like Growth Factor-I/Recombinant Human Insulin-Like Growth Factor Binding Protein-3 (rhIGF-I/rhIGFBP-3) In Myotonic Dystrophy Type 1

Myotonic Dystrophy Type 1

Enrolled (actual)
69
Serious AEs
18.8%
Results posted
Jan 2022
Primary outcome: Primary: Change From Baseline to Week 24 in Distance Walked as Assessed by the Six-minute Walk Test (6MWT) Distance — 12.40; 20.11 meters

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
rhIGF-I/rhIGFBP-3 (Drug); placebo (Drug)
Age
Adult, Older Adult · 21+ yrs
Sex
All
Sponsor
Insmed Incorporated
Primary completion
Dec 2008

Outcome Measures

OutcomeResultp-value
PRIMARY
Change From Baseline to Week 24 in Distance Walked as Assessed by the Six-minute Walk Test (6MWT) Distance
12.40; 20.11
PRIMARY
Change From Baseline in Daily Step Count
-140; -58
PRIMARY
Peak Activity Index: Change From Baseline in Number of Steps Walked Per Minute During the 30 Minute Period of Fastest Walking
0.5; 1.1
PRIMARY
Sustained Activity Index: Change From Baseline in the Highest Number of Steps Walked Per Minute Over 20 Minutes of Activity
-0.9; 0.8
PRIMARY
Change From Baseline in the Percentage of Time That Participants Spent Inactive
-0.4; -0.0
PRIMARY
Change From Baseline in Time Taken for Participants to Ascend and Descend 4 Stairs
0.0; -0.5; -0.1; -0.5
PRIMARY
Change From Baseline in Time Taken to Traverse 30 Feet
0.2; -0.2
PRIMARY
Change From Baseline in Purdue Pegboard Test Scores
-0.2; 0.3
PRIMARY
Change From Baseline in Forced Vital Capacity (FVC) Volume While Sitting or Lying Down
-0.1; 0.0; -0.2; -0.1
PRIMARY
Change From Baseline in Forced Vital Capacity (FVC) Percent Predicted While Sitting or Lying Down
0.0; -2.3; -3.8; -3.2
PRIMARY
Change From Baseline in Manual Muscle Test (MMT) Scores
0.9; -0.3; -0.4; 0.0; 1.0; -0.3
PRIMARY
Change From Baseline in Selective Reminding Test T-Scores (Total Word and Delayed Words)
4.3; 2.9; -3.2; 0.04
PRIMARY
Change From Baseline in Selective Reminding Test Raw Scores (Cued Recall and Recognition)
0.0; -0.4; 0.0; 0
PRIMARY
Change From Baseline in Rey Complex Figure (RCF) Test Scores
7.5; 4.8; 5.2; 4.8; -1.5; 5.9
PRIMARY
Change From Baseline in Letter-Number Sequencing (LNS) Test Scores
1.18; -0.36
PRIMARY
Change From Baseline in Trail Making Test (TMT) Scores
2.2; -0.3; 4.0; -0.9
PRIMARY
Change From Baseline in the Stroop Color Word Test Scores
-0.4; 2.0; 2.0; 0.9; 1.3; 1.0
PRIMARY
Change From Baseline to Week 24 Scores on the Beck Depression Inventory II (BDI-II) Questionnaire
-1.63; -0.14
PRIMARY
Change From Baseline in Average Fasting Glucose Concentration in the Blood
-6.8; 0.2
PRIMARY
Change From Baseline in Average Fasting Insulin Concentration in the Blood
-5.7; 0.7
PRIMARY
Change From Baseline in Qualitative Insulin Sensitivity Check Index (QUICKI)
0.05; -0.01
PRIMARY
Change From Baseline in Insulin Sensitivity Index-Matsuda (ISI-Matsuda)
5.5; -1.4
PRIMARY
Change From Baseline in Total Blood Cholesterol Level
-7.9; -2.7
PRIMARY
Change From Baseline in Total Blood Low-density Lipoproteins (LDL) Level
1.5; 5.0
PRIMARY
Change From Baseline in Total Blood High-density Lipoproteins (HDL) Level
0.3; -3.0
PRIMARY
Change From Baseline in Total Blood Triglycerides Level
-54.8; -21.7
PRIMARY
Change From Baseline in Gastro-esophageal Reflux Disease (GERD) Symptom Frequency Questionnaire (GSFQ) Scores
-6.4; -0.5
PRIMARY
Change From Baseline in Gastrointestinal Symptom Rating Scale for Irritable Bowel Syndrome (GSRS-IBS) Questionnaire Scores
-0.29; -0.02
PRIMARY
Change From Baseline in Swallowing Disturbance Questionnaire (SDQ) Scores
0.29; -0.34
PRIMARY
Change From Baseline in Short Form (36) (SF-36) Questionnaire Scores
1.4; -0.5; 1.2; 1.2
PRIMARY
Change From Baseline in Brief Pain Inventory (BPI) Questionnaire - Severity Scores
0.0; 0.5; -0.2; 0.6
PRIMARY
Change From Baseline in Brief Pain Inventory (BPI) Questionnaire - Interference Scores
0.0; -0.5

Summary

To investigate the effects of rhIGF-I/rhIGFBP-3 treatment for 24 weeks on endurance, ambulation, cognitive functioning, insulin resistance, lipid levels, muscle function and strength, pain, gastrointestinal functioning, and quality of life endpoints in DM1 patients

Eligibility Criteria

Inclusion Criteria (list is not inclusive):

  • A diagnosis of DM1, confirmed by DM1 genetic mutation
  • Ability to walk 30 feet - assistance with cane and/or leg bracing permitted
  • Able to self-administer study medication by subcutaneous injection or caregiver is available to administer study medication

Exclusion Criteria (list is not inclusive):

  • Congenital DM1
  • Weight greater than 100 kg or body mass index greater than 30 kg/m2
  • Prior treatment with glucocorticoids, anabolic steroids, testosterone, growth hormone, investigational agent within 60 days of screening
  • Current diagnosis or history of malignancy expect for surgically cured skin cancer or pilomatricoma
  • Changes in lipid lowering medications during the 3 months prior to screening
  • Diaphragmatic weakness such that patients are unable to tolerate the supine position, or swallowing impairment such that patients are unable to maintain nutrition without use of gastrostomy.
  • Major psychiatric illness (major depression, bipolar disorder or schizophrenia) within twelve months of screening
  • History of non-compliance with other therapies
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00577577). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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