Trial Outcomes & Findings for Phase III Trial of Coenzyme Q10 in Mitochondrial Disease (NCT NCT00432744)

NCT ID: NCT00432744

Last Updated: 2017-09-11

Results Overview

The McMaster Gross Motor Function is a validated scale ranging from 0 to 100 (the higher the better). Since there was the possibility of a subject becoming totally disabled our FDA peer reviewed design called for its use as follows: If the subject completed both periods, the score was calculated as the difference in scores between the end of Period 2 (at 12 months) minus that at the end of Period 1 (6 months). If a subject became totally disabled, this difference was considered as plus infinity if it occurred in period 1 (Penalizes period 1), and minus infinity if it occurred in Period 2 (Penalizes period 2). The two treatments were compared via the Wilcoxon test, and the effect size was estimated using Kendall's Tau-B. This is interpreted in a similar manner to correlation with positive values favoring COQenzyme10 and negative values favoring placebo. One of the links in this report is to the the GMFM scale and how it is scored. A link to the instrument is included.

Recruitment status

COMPLETED

Study phase

PHASE3

Target enrollment

24 participants

Primary outcome timeframe

Taken at 6 and 12 Months

Results posted on

2017-09-11

Participant Flow

Participant milestones

Participant milestones
Measure
Placebo First
Patients will be randomized to receive Placebo in Period #1 (Months 0-6) and CoenzymeQ10 given in Period #2( Months 7-12.) Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group. CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed.
CoenzymeQ10 Frist
Patients will be randomized to receive CoenzymeQ10 in Period #1 (Months 0-6) and Placebo given in Period #2( Months 7-12.) CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed. Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group.
Months 0-6
STARTED
12
12
Months 0-6
COMPLETED
7
8
Months 0-6
NOT COMPLETED
5
4
Months 7 - 12
STARTED
7
8
Months 7 - 12
COMPLETED
6
7
Months 7 - 12
NOT COMPLETED
1
1

Reasons for withdrawal

Reasons for withdrawal
Measure
Placebo First
Patients will be randomized to receive Placebo in Period #1 (Months 0-6) and CoenzymeQ10 given in Period #2( Months 7-12.) Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group. CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed.
CoenzymeQ10 Frist
Patients will be randomized to receive CoenzymeQ10 in Period #1 (Months 0-6) and Placebo given in Period #2( Months 7-12.) CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed. Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group.
Months 0-6
Withdrawal by Subject
4
4
Months 0-6
Too disabled to score
1
0
Months 7 - 12
Withdrawal by Subject
1
0
Months 7 - 12
Too disabled to score
0
1

Baseline Characteristics

Phase III Trial of Coenzyme Q10 in Mitochondrial Disease

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Placebo First
n=12 Participants
Patients will be randomized to receive Placebo in Period #1 (Months 0-6) and CoenzymeQ10 given in Period #2( Months 7-12.) Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group. CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed.
CoenzymeQ10 Frist
n=12 Participants
Patients will be randomized to receive CoenzymeQ10 in Period #1 (Months 0-6) and Placebo given in Period #2( Months 7-12.) CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed. Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group.
Total
n=24 Participants
Total of all reporting groups
Age, Continuous
9.0 years
STANDARD_DEVIATION 6.3 • n=99 Participants
7.5 years
STANDARD_DEVIATION 4.5 • n=107 Participants
8.3 years
STANDARD_DEVIATION 5.4 • n=206 Participants
Age, Categorical
<=18 years
12 Participants
n=99 Participants
12 Participants
n=107 Participants
24 Participants
n=206 Participants
Age, Categorical
Between 18 and 65 years
0 Participants
n=99 Participants
0 Participants
n=107 Participants
0 Participants
n=206 Participants
Age, Categorical
>=65 years
0 Participants
n=99 Participants
0 Participants
n=107 Participants
0 Participants
n=206 Participants
Sex: Female, Male
Female
6 Participants
n=99 Participants
8 Participants
n=107 Participants
14 Participants
n=206 Participants
Sex: Female, Male
Male
6 Participants
n=99 Participants
4 Participants
n=107 Participants
10 Participants
n=206 Participants
Region of Enrollment
United States
10 participants
n=99 Participants
9 participants
n=107 Participants
19 participants
n=206 Participants
Region of Enrollment
Canada
2 participants
n=99 Participants
3 participants
n=107 Participants
5 participants
n=206 Participants

PRIMARY outcome

Timeframe: Taken at 6 and 12 Months

The McMaster Gross Motor Function is a validated scale ranging from 0 to 100 (the higher the better). Since there was the possibility of a subject becoming totally disabled our FDA peer reviewed design called for its use as follows: If the subject completed both periods, the score was calculated as the difference in scores between the end of Period 2 (at 12 months) minus that at the end of Period 1 (6 months). If a subject became totally disabled, this difference was considered as plus infinity if it occurred in period 1 (Penalizes period 1), and minus infinity if it occurred in Period 2 (Penalizes period 2). The two treatments were compared via the Wilcoxon test, and the effect size was estimated using Kendall's Tau-B. This is interpreted in a similar manner to correlation with positive values favoring COQenzyme10 and negative values favoring placebo. One of the links in this report is to the the GMFM scale and how it is scored. A link to the instrument is included.

Outcome measures

Outcome measures
Measure
Placebo First
n=6 Participants
Patients will be randomized to receive Placebo in Period #1 (Months 0-6) and CoenzymeQ10 given in Period #2( Months 7-12.) Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group. CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed.
CoenzymeQ10 Frist
n=8 Participants
Patients will be randomized to receive CoenzymeQ10 in Period #1 (Months 0-6) and Placebo given in Period #2( Months 7-12.) CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed. Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group.
McMaster Gross Motor Function (GMFM 88)
-0.002 units on a scale
Interval -0.47 to 1.36
-0.12 units on a scale
Interval -2.75 to 1.75

PRIMARY outcome

Timeframe: At 6 and 12 Months

Population: 7 subjects in each group were evaluable for this endpoint. The two subjects who became disabled are evaluable. One subject did not have period 2 Quality of Life data and is excluded.

The Pediatric Quality of Life Scale is a validated scale ranging from 0 to 100 (the higher the better). Since there was the possibility of a subject becoming totally disabled our FDA peer reviewed design called for its use as follows: If the subject completed both periods, the score was calculated as the difference in scores between the end of Period 2 (at 12 months) minus that at the end of Period 1 (6 months). If a subject became totally disabled, this difference was considered as plus infinity if it occurred in period 1 (Penalizes period 1), and minus infinity if it occurred in Period 2 (Penalizes period 2). The two treatments were compared via the Wilcoxon test, and the effect size was estimated using Kendall's Tau-B. This is interpreted in a similar manner to correlation with positive values favoring COQenzyme10 and negative values favoring placebo. Goggle "pedsQL and Mapi" to browse the copyrighted manual. A link to the instrument is included.

Outcome measures

Outcome measures
Measure
Placebo First
n=7 Participants
Patients will be randomized to receive Placebo in Period #1 (Months 0-6) and CoenzymeQ10 given in Period #2( Months 7-12.) Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group. CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed.
CoenzymeQ10 Frist
n=7 Participants
Patients will be randomized to receive CoenzymeQ10 in Period #1 (Months 0-6) and Placebo given in Period #2( Months 7-12.) CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed. Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group.
Pediatric Quality of Life Scale
-1.1 units on a scale
Interval -4.3 to 7.1
-11.9 units on a scale
Interval -43.5 to 4.3

PRIMARY outcome

Timeframe: end of 12 month minus end of 6 month difference.

Population: Subjects contributes at least one difference (either GMFM or Peds QOL). Thirteen contributed both and one each was missing GMFM or Peds QOL.

This is a multivariate analysis of the first two outcomes: Period 2 minus Period 1 GMFM88 and Peds Quality of Life, analyzed as follows: First, to be in the analysis, subjects must contribute at least one of these endpoints. Second, if the subject became totally disabled during period 1, the difference was defined as + infinity, (highest possible evidence favoring period 2), and if the subject became totally disabled in period 2, the subject was scored as - infinity (highest possible evidence favoring period 1). Period 2 minus period 1 differences were ranked form low to high with missing values scores at the mid-rank. The Hotelling T-square was computed on these ranks and the P-value was obtained from 100,000 rerandomizations as the fraction of rerandomizations with T-sq at least as large as that observed.

Outcome measures

Outcome measures
Measure
Placebo First
n=7 Participants
Patients will be randomized to receive Placebo in Period #1 (Months 0-6) and CoenzymeQ10 given in Period #2( Months 7-12.) Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group. CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed.
CoenzymeQ10 Frist
n=8 Participants
Patients will be randomized to receive CoenzymeQ10 in Period #1 (Months 0-6) and Placebo given in Period #2( Months 7-12.) CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed. Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group.
Non-parametric Hotelling T-square Bivariate Analysis of GMGF 88 and OPeds QOL.
7 participants
8 participants

Adverse Events

CoenzymeQ10

Serious events: 1 serious events
Other events: 10 other events
Deaths: 0 deaths

Placebo

Serious events: 2 serious events
Other events: 10 other events
Deaths: 0 deaths

Serious adverse events

Serious adverse events
Measure
CoenzymeQ10
n=19 participants at risk
CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed. (Either in Period 1 or Period 2)
Placebo
n=20 participants at risk
Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group. (Either in Period 1 or Period 2)
Musculoskeletal and connective tissue disorders
Hospitalization for lumbar vertebrae fractures
0.00%
0/19 • AE's were collected from 10/2007 to 4/2012.
5.0%
1/20 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
Nervous system disorders
Altered Mental State
0.00%
0/19 • AE's were collected from 10/2007 to 4/2012.
5.0%
1/20 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
Nervous system disorders
Hospitalization for seizure activity
5.3%
1/19 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
0.00%
0/20 • AE's were collected from 10/2007 to 4/2012.

Other adverse events

Other adverse events
Measure
CoenzymeQ10
n=19 participants at risk
CoenzymeQ10: CoenzymeQ10 will be given in 10 mg/kg daily up to 400 mg. Then a draw of CoQ10 troughs every three months will be performed. (Either in Period 1 or Period 2)
Placebo
n=20 participants at risk
Placebo: Placebo will be given in 10 mg/kg daily up to 400 mg. Then a draw of placebo troughs every three months will be performed. This treatment group will be treated as the active group. (Either in Period 1 or Period 2)
Blood and lymphatic system disorders
Decreased White Blood Count
5.3%
1/19 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
0.00%
0/20 • AE's were collected from 10/2007 to 4/2012.
Blood and lymphatic system disorders
Low ANC Levels
0.00%
0/19 • AE's were collected from 10/2007 to 4/2012.
5.0%
1/20 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
General disorders
Nose Bleed
0.00%
0/19 • AE's were collected from 10/2007 to 4/2012.
5.0%
1/20 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
Blood and lymphatic system disorders
Elevated Liver Enzymes
5.3%
1/19 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
5.0%
1/20 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
Ear and labyrinth disorders
Ear Infection
5.3%
1/19 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
5.0%
1/20 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
Gastrointestinal disorders
GI Issues
10.5%
2/19 • Number of events 2 • AE's were collected from 10/2007 to 4/2012.
25.0%
5/20 • Number of events 5 • AE's were collected from 10/2007 to 4/2012.
General disorders
Fatigue
5.3%
1/19 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
0.00%
0/20 • AE's were collected from 10/2007 to 4/2012.
Musculoskeletal and connective tissue disorders
Neuromusclar Changes
5.3%
1/19 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
5.0%
1/20 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
Nervous system disorders
Mental Status Change
5.3%
1/19 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
0.00%
0/20 • AE's were collected from 10/2007 to 4/2012.
Skin and subcutaneous tissue disorders
Rash
5.3%
1/19 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
0.00%
0/20 • AE's were collected from 10/2007 to 4/2012.
Infections and infestations
GTube Infection
5.3%
1/19 • Number of events 1 • AE's were collected from 10/2007 to 4/2012.
0.00%
0/20 • AE's were collected from 10/2007 to 4/2012.

Additional Information

Dr. Peter W Stacpoole

University of Florida

Phone: 352-273-9023

Results disclosure agreements

  • Principal investigator is a sponsor employee
  • Publication restrictions are in place