Trial Outcomes & Findings for Fatty Acid Oxidation Disorders & Body Weight Regulation Grant (NCT NCT00654004)

NCT ID: NCT00654004

Last Updated: 2013-04-22

Results Overview

Body composition by DEXA was measured in subjects with a long-chain fatty acid oxidation disorder (n=13). Twelve age, sex and BMI matched controls and 4 heterozygotes for a long-chain fatty acid oxidation disorder were recruited who also completed body composition measures. The difference in body composition between subjects and age matched controls was compared by t-test.

Recruitment status

COMPLETED

Target enrollment

26 participants

Primary outcome timeframe

Subjects will be compared to controls at one point in time.

Results posted on

2013-04-22

Participant Flow

Subjects with a long-chain fatty acid oxidation disorder were recruited through announcements on disease specific websites, and referrals from metabolic physicians. Controls were recruited through the OHSU website and word of mouth.

After a subject had completed the protocol, potential control subjects were screened for age, gender and BMI that would allow for a 1 to 1 matching study design.

Participant milestones

Participant milestones
Measure
Subjects
Subjects are patients with a long-chain fatty acid oxidation disorder including CPT2, VLCAD, TFP or LCHAD deficiency.
Controls
Subjects do not have a fatty acid oxidation disorder.
Overall Study
STARTED
14
12
Overall Study
COMPLETED
13
12
Overall Study
NOT COMPLETED
1
0

Reasons for withdrawal

Reasons for withdrawal
Measure
Subjects
Subjects are patients with a long-chain fatty acid oxidation disorder including CPT2, VLCAD, TFP or LCHAD deficiency.
Controls
Subjects do not have a fatty acid oxidation disorder.
Overall Study
Lost to Follow-up
1
0

Baseline Characteristics

Fatty Acid Oxidation Disorders & Body Weight Regulation Grant

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Subjects
n=14 Participants
Subjects are patients with a long-chain fatty acid oxidation disorder including CPT2, VLCAD, TFP or LCHAD deficiency.
Controls
n=12 Participants
Subjects do not have a fatty acid oxidation disorder.
Total
n=26 Participants
Total of all reporting groups
Age, Categorical
<=18 years
11 Participants
n=99 Participants
10 Participants
n=107 Participants
21 Participants
n=206 Participants
Age, Categorical
Between 18 and 65 years
3 Participants
n=99 Participants
2 Participants
n=107 Participants
5 Participants
n=206 Participants
Age, Categorical
>=65 years
0 Participants
n=99 Participants
0 Participants
n=107 Participants
0 Participants
n=206 Participants
Age Continuous
14 years
STANDARD_DEVIATION 8 • n=99 Participants
15 years
STANDARD_DEVIATION 7 • n=107 Participants
14.3 years
STANDARD_DEVIATION 7.7 • n=206 Participants
Sex: Female, Male
Female
6 Participants
n=99 Participants
5 Participants
n=107 Participants
11 Participants
n=206 Participants
Sex: Female, Male
Male
8 Participants
n=99 Participants
7 Participants
n=107 Participants
15 Participants
n=206 Participants
Region of Enrollment
United States
12 participants
n=99 Participants
12 participants
n=107 Participants
24 participants
n=206 Participants
Region of Enrollment
Canada
1 participants
n=99 Participants
0 participants
n=107 Participants
1 participants
n=206 Participants
Region of Enrollment
Europe
1 participants
n=99 Participants
0 participants
n=107 Participants
1 participants
n=206 Participants

PRIMARY outcome

Timeframe: Subjects will be compared to controls at one point in time.

Population: Study design was based on 1 to 1 matching of subjects and controls. Thirteen subjects but only 12 controls completed the protocol. We report results of 12 subjects compared to 12 matched controls.

Body composition by DEXA was measured in subjects with a long-chain fatty acid oxidation disorder (n=13). Twelve age, sex and BMI matched controls and 4 heterozygotes for a long-chain fatty acid oxidation disorder were recruited who also completed body composition measures. The difference in body composition between subjects and age matched controls was compared by t-test.

Outcome measures

Outcome measures
Measure
Subjects
n=12 Participants
Subjects are patients with a long-chain fatty acid oxidation disorder including CPT2, VLCAD, TFP or LCHAD deficiency.
Controls
n=12 Participants
Subjects do not have a fatty acid oxidation disorder.
An Outcome of This Study is the Difference in Percent Body Fat (%BF) Between Subjects With a Long-chain Fatty Acid Oxidation Disorder and Normal Controls.
31.8 percentage of body fat
Standard Deviation 6.8
27.2 percentage of body fat
Standard Deviation 9.1

PRIMARY outcome

Timeframe: Subjects will be compared to controls at one point in time.

Population: Study design was based on 1 to 1 matching of subjects and controls. Thirteen subjects but only 12 controls completed the protocol. We report results of 12 subjects compared to 12 matched controls.

Glucose tolerance was estimated by the Matsuda Index using glucose and insulin values from a standard oral glucose tolerance test. The Matsuda Index is calculated by the following formula: 10,000/ sq root of (fasting glucose mg/dl X fasting insulin in units/ml) X (mean glucose (mg/dl) X mean insulin (units/ml) and correlates with insulin sensitivity measured by the gold standard method of a hyperinsulinemic euglycemic clamp. Values of 2.5 or greater are considered insulin sensitive. Values of 2.4 or less are considered insulin resistance. The Matsuda Index of Insulin Sensitivity was measured in subjects with a long-chain fatty acid oxidation disorder (n=12). Twelve age, sex and BMI matched controls and 4 heterozygotes for a long-chain fatty acid oxidation disorder were recruited who also completed an oral glucose tolerance test. The difference in Mastuda Index between subjects and age matched controls was compared by t-test.

Outcome measures

Outcome measures
Measure
Subjects
n=12 Participants
Subjects are patients with a long-chain fatty acid oxidation disorder including CPT2, VLCAD, TFP or LCHAD deficiency.
Controls
n=12 Participants
Subjects do not have a fatty acid oxidation disorder.
An Outcome of This Study is the Difference in Glucose Tolerance Between Subjects With a Long-chain Fatty Acid Oxidation Disorder and Normal Controls.
2.8 units on a scale
Standard Deviation 1.8
3.13 units on a scale
Standard Deviation 1.57

SECONDARY outcome

Timeframe: Fasting total adiponectin (ug/ml)

Population: Study was designed as a one to one matching design. Thirteen subjects but only 12 controls completed the protocol. We report results of 12 subjects and 12 matched controls.

Fasting total adiponectin levels in ug/ml were measured in both groups (subjects with a long-chain fatty acid oxidation disorder). The differences between groups were compared with a t-test

Outcome measures

Outcome measures
Measure
Subjects
n=12 Participants
Subjects are patients with a long-chain fatty acid oxidation disorder including CPT2, VLCAD, TFP or LCHAD deficiency.
Controls
n=12 Participants
Subjects do not have a fatty acid oxidation disorder.
The Difference in Plasma Adiponectin Levels Between Subjects With a Long-chain Fatty Acid Oxidation Disorder and Matched Controls Was Compared by T-test
17.3 ug/ml
Standard Deviation 7.2
24.4 ug/ml
Standard Deviation 13.5

SECONDARY outcome

Timeframe: Fasting leptin levels ng per kg of fat mass

Population: Study was designed as a one to one matching design. Thirteen subjects but only 12 controls completed the protocol. We report results of 12 subjects and 12 matched controls.

Fasting leptin in ng/kg fat mass were measured in both groups (subjects with a long-chain fatty acid oxidation disorder; controls). The differences between groups were compared with a t-test

Outcome measures

Outcome measures
Measure
Subjects
n=12 Participants
Subjects are patients with a long-chain fatty acid oxidation disorder including CPT2, VLCAD, TFP or LCHAD deficiency.
Controls
n=12 Participants
Subjects do not have a fatty acid oxidation disorder.
The Difference in Plasma Leptin Between Subjects With a Long-chain Fatty Acid Oxidation Disorder and Matched Controls Was Compared by T-test
0.99 ng/kg
Standard Deviation 0.8
0.91 ng/kg
Standard Deviation 0.57

SECONDARY outcome

Timeframe: Fasting insulin levels uUnits/ml

Population: Study was designed as a one to one matching design. Thirteen subjects but only 12 controls completed the protocol. Samples were missing on one subject so 11 subjects were compared to 11 controls.

Fasting insulin levels in uU/ml were measured in both groups. The differences between groups were compared with a t-test

Outcome measures

Outcome measures
Measure
Subjects
n=11 Participants
Subjects are patients with a long-chain fatty acid oxidation disorder including CPT2, VLCAD, TFP or LCHAD deficiency.
Controls
n=11 Participants
Subjects do not have a fatty acid oxidation disorder.
The Difference in Plasma Insulin Between Subjects With a Long-chain Fatty Acid Oxidation Disorder and Matched Controls Was Compared by T-test
17 uU/ml
Standard Deviation 8
13 uU/ml
Standard Deviation 5

Adverse Events

Subjects

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

Controls

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

Serious adverse events

Adverse event data not reported

Other adverse events

Adverse event data not reported

Additional Information

Dr. Melanie Gillingham

Oregon Health & Science Univeristy

Phone: 503-494-1682

Results disclosure agreements

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